Neuro Flashcards
Q: What is Wernicke’s (receptive) aphasia and its cause?
A: Wernicke’s aphasia is caused by a lesion of the superior temporal gyrus, typically supplied by the inferior division of the left MCA. It results in fluent speech that makes no sense, with word substitutions and neologisms, but impaired comprehension.
Q: What characterizes Broca’s (expressive) aphasia?
A: Broca’s aphasia is characterized by non-fluent, laboured, and halting speech with impaired repetition but normal comprehension. It is caused by a lesion of the inferior frontal gyrus, typically supplied by the superior division of the left MCA.
Q: What is conduction aphasia and what causes it?
A: Conduction aphasia is caused by a stroke affecting the arcuate fasciculus, the connection between Wernicke’s and Broca’s areas. It results in fluent speech with poor repetition, but normal comprehension and awareness of errors.
Q: Describe global aphasia.
A: Global aphasia results from a large lesion affecting Wernicke’s, Broca’s, and the arcuate fasciculus areas, leading to severe expressive and receptive aphasia. Patients may still be able to communicate using gestures.
Q: What is the key difference between dysarthria and aphasia?
A: Dysarthria refers to a motor speech disorder affecting the muscles used in speech, while aphasia is a language disorder affecting speech production, comprehension, or both due to brain lesions.
Q: What are 5-HT3 antagonists primarily used for?
A: They are antiemetics used mainly in the management of chemotherapy-related nausea.
Q: Where do 5-HT3 antagonists mainly act?
A: They mainly act in the chemoreceptor trigger zone area of the medulla oblongata.
Q: Name two examples of 5-HT3 antagonists.
A: Ondansetron and palonosetron.
Q: What is the main advantage of second-generation 5-HT3 antagonists like palonosetron?
A: They have a reduced effect on the QT interval.
Q: What is a common adverse effect of 5-HT3 antagonists?
A: Constipation.
Q: What is a significant adverse effect associated with 5-HT3 antagonists?
A: Prolonged QT interval.
Q: What type of ataxia is caused by cerebellar hemisphere lesions?
A: Cerebellar hemisphere lesions cause peripheral ataxia, also known as ‘finger-nose ataxia’.
Q: What type of ataxia is associated with cerebellar vermis lesions?
A: Cerebellar vermis lesions cause gait ataxia.
Q: What is the genetic cause of ataxia telangiectasia?
A: Ataxia telangiectasia is caused by a defect in the ATM gene, which encodes for DNA repair enzymes.
Q: How is ataxia telangiectasia inherited?
A: Ataxia telangiectasia is inherited in an autosomal recessive manner.
Q: When does ataxia telangiectasia typically present?
A: It typically presents in early childhood with abnormal movements.
Q: List the main features of ataxia telangiectasia.
A: The main features include cerebellar ataxia, telangiectasia (spider angiomas), IgA deficiency with recurrent chest infections, and a 10% risk of developing malignancy (such as lymphoma or leukemia), as well as non-lymphoid tumors.
Q: What immunodeficiency is associated with ataxia telangiectasia?
A: IgA deficiency, which results in recurrent chest infections.
Q: What is autonomic dysreflexia?
A: Autonomic dysreflexia is a clinical syndrome occurring in patients with a spinal cord injury at or above the T6 spinal level, characterized by extreme hypertension and other autonomic disturbances.
Q: What are common triggers for autonomic dysreflexia?
A: Common triggers include faecal impaction and urinary retention, though many other triggers have been reported.
Q: What physiological response occurs in autonomic dysreflexia?
A: Afferent signals trigger a sympathetic spinal reflex via thoracolumbar outflow, but the usual centrally mediated parasympathetic response is prevented by the cord lesion.
Q: What are the main features of autonomic dysreflexia?
A: The main features include extreme hypertension, flushing and sweating above the level of the cord lesion, and agitation.
Q: What are the severe consequences of untreated autonomic dysreflexia?
A: Untreated cases can lead to severe consequences such as haemorrhagic stroke due to extreme hypertension.
Q: What is the management of autonomic dysreflexia?
A: Management involves removal or control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.
Q: What is Bell’s palsy?
A: Bell’s palsy is an acute, unilateral, idiopathic facial nerve paralysis.
Q: What is the suspected etiology of Bell’s palsy?
A: The exact etiology is unknown, but the herpes simplex virus has been investigated as a possible cause.
Q: What is the peak incidence age range for Bell’s palsy?
A: The peak incidence is between 20-40 years.
Q: Which group is more commonly affected by Bell’s palsy?
A: Bell’s palsy is more common in pregnant women.
Q: What are the main features of Bell’s palsy?
A: Features include lower motor neuron facial nerve palsy affecting the forehead, post-auricular pain, altered taste, dry eyes, and hyperacusis.
Q: What is the key distinguishing feature between lower motor neuron and upper motor neuron facial palsy?
A: Lower motor neuron facial palsy affects the forehead, whereas upper motor neuron lesions spare the upper face.
Q: What is the recommended initial management for Bell’s palsy?
A: All patients should receive oral prednisolone within 72 hours of onset.
Q: Is the use of antivirals recommended in the treatment of Bell’s palsy?
A: The addition of antivirals is debated, but UpToDate recommends their use for severe facial palsy. Antiviral treatments alone are not recommended.
Q: Why is eye care important in Bell’s palsy, and what measures should be taken?
A: Eye care is important to prevent exposure keratopathy. Measures include prescribing artificial tears, eye lubricants, and taping the eye closed with microporous tape at bedtime if necessary.
Q: When should a patient with Bell’s palsy be referred to ENT?
A: Patients should be referred urgently to ENT if there is no sign of improvement after 3 weeks.
Q: When might a referral to plastic surgery be appropriate for Bell’s palsy patients?
A: A referral to plastic surgery may be appropriate for patients with more long-standing weakness, such as several months.
Q: What is the prognosis for most people with Bell’s palsy?
A: Most people make a full recovery within 3-4 months.
Q: What percentage of untreated Bell’s palsy patients have permanent moderate to severe weakness?
A: Around 15% of untreated patients have permanent moderate to severe weakness.
Q: What is Erb-Duchenne paralysis, and what causes it?
A: Erb-Duchenne paralysis is caused by damage to the C5 and C6 roots of the brachial plexus. It may result from a breech presentation during birth and is characterized by weakness or paralysis in the shoulder and upper arm muscles.
Q: What is a common sign of Erb-Duchenne paralysis?
A: A common sign is a “winged scapula,” where the scapula protrudes abnormally due to weakness in the muscles controlling it.
Q: What is Klumpke’s paralysis, and what causes it?
A: Klumpke’s paralysis is caused by damage to the T1 root of the brachial plexus, often due to traction. It results in the loss of intrinsic hand muscles and may cause claw hand deformity.
Q: What is a characteristic feature of Klumpke’s paralysis?
A: The loss of intrinsic hand muscles, leading to difficulty with fine motor control and possibly claw hand deformity.
Q: What are the common causes of brain abscesses?
A: Brain abscesses can result from middle ear or sinus sepsis, trauma or surgery to the scalp, penetrating head injuries, and embolic events from endocarditis.
Q: What are the common presenting symptoms of a brain abscess?
A: Symptoms may include headache (often dull and persistent), fever (which may be absent), focal neurology (e.g., oculomotor nerve palsy), raised intracranial pressure (leading to nausea, papilloedema, seizures), and other neurological deficits.
Q: What is a typical finding on imaging for brain abscesses?
A: CT scanning is used to assess brain abscesses, revealing the abscess cavity and any mass effect.
Q: What is the main management for brain abscesses?
A: Management includes surgery (craniotomy for abscess drainage), IV antibiotics (e.g., 3rd-generation cephalosporins + metronidazole), and intracranial pressure management (e.g., dexamethasone).
Q: Why might a craniotomy not fully prevent abscess recurrence?
A: The abscess may reform because the head is closed following drainage, which may limit further drainage of residual infection.
Q: What are the main features of parietal lobe lesions?
A: Features of parietal lobe lesions include sensory inattention, apraxias, astereognosis (tactile agnosia), inferior homonymous quadrantanopia, and Gerstmann’s syndrome (alexia, acalculia, finger agnosia, right-left disorientation).
Q: What is the effect of occipital lobe lesions?
A: Occipital lobe lesions can lead to homonymous hemianopia (with macula sparing), cortical blindness, and visual agnosia.
Q: What are the signs of a temporal lobe lesion?
A: Temporal lobe lesions can cause Wernicke’s aphasia (fluent but nonsensical speech with word substitutions and neologisms), superior homonymous quadrantanopia, auditory agnosia, and prosopagnosia (difficulty recognizing faces).
Q: What are the effects of frontal lobe lesions?
A: Frontal lobe lesions can cause expressive (Broca’s) aphasia (non-fluent, laboured speech), disinhibition, perseveration, anosmia, and inability to generate a list.
Q: What are the effects of cerebellum lesions?
A: Midline cerebellar lesions cause gait and truncal ataxia, while hemisphere lesions lead to intention tremor, past pointing, dysdiadokinesis, and nystagmus.
Q: What conditions are associated with the medial thalamus and mammillary bodies of the hypothalamus?
A: Lesions in these areas are associated with Wernicke and Korsakoff syndrome.
Q: What condition is linked with a lesion in the subthalamic nucleus of the basal ganglia?
A: Lesions in the subthalamic nucleus are associated with hemiballism.
Q: What is the result of a lesion in the striatum (caudate nucleus) of the basal ganglia?
A: Lesions in the striatum are associated with Huntington’s chorea.
Q: What condition is caused by damage to the substantia nigra of the basal ganglia?
A: Lesions in the substantia nigra lead to Parkinson’s disease.
Q: What is Kluver-Bucy syndrome, and which brain area is involved?
A: Kluver-Bucy syndrome, associated with lesions in the amygdala, causes hypersexuality, hyperorality, hyperphagia, and visual agnosia.
Q: What is the most common type of brain tumour in adults?
A: The most common primary brain tumour in adults is glioblastoma multiforme. It is associated with a poor prognosis (~1 year) and presents as a solid tumour with central necrosis and contrast enhancement.
Q: What is the typical presentation and treatment of meningiomas?
A: Meningiomas are benign, extrinsic tumours arising from the arachnoid cap cells of the meninges. They typically present with symptoms caused by compression of nearby structures. Treatment involves observation, radiotherapy, or surgical resection.
Q: What is a vestibular schwannoma and its association with neurofibromatosis?
A: A vestibular schwannoma (acoustic neuroma) is a benign tumour of the eighth cranial nerve, commonly presenting with hearing loss, tinnitus, and facial nerve palsy. It is associated with neurofibromatosis type 2, which causes bilateral vestibular schwannomas.
Q: What is the most common primary brain tumour in children?
A: The most common primary brain tumour in children is pilocytic astrocytoma. It is often diagnosed in childhood and presents with symptoms related to the location of the tumour.
Q: What is a medulloblastoma and its typical treatment?
A: A medulloblastoma is an aggressive paediatric brain tumour arising from the infratentorial compartment. It spreads through the CSF system, and treatment usually involves surgical resection and chemotherapy.
Q: What is the typical location and treatment of an ependymoma?
A: Ependymomas are commonly found in the 4th ventricle and may cause hydrocephalus. Treatment is typically surgical, and the tumour can be diagnosed by its histology showing perivascular pseudorosettes.
Q: What is an oligodendroma, and where is it commonly located?
A: An oligodendroma is a benign, slow-growing tumour commonly found in the frontal lobes. It presents with calcifications that give a ‘fried-egg’ appearance on histology.
Q: What is the characteristic feature of a haemangioblastoma?
A: Haemangioblastomas are vascular tumours of the cerebellum, often associated with von Hippel-Lindau syndrome. They are characterized by foam cells and high vascularity on histology.
Q: What are the different types of pituitary adenomas?
A: Pituitary adenomas can be secretory (e.g., Cushing’s syndrome or acromegaly) or non-secretory. They can also be microadenomas (less than 1 cm) or macroadenomas (greater than 1 cm). They may cause visual disturbances like bitemporal hemianopia due to optic chiasm compression.
Q: What is a craniopharyngioma and how is it treated?
A: A craniopharyngioma is a solid/cystic tumour arising from the remnants of Rathke’s pouch. It is common in children and may present with hormonal disturbances, hydrocephalus, or bitemporal hemianopia. Treatment is typically surgical with or without postoperative radiotherapy.
Q: What is the cause of Brown-Sequard syndrome?
A: Brown-Sequard syndrome is caused by a lateral hemisection (or damage) of the spinal cord.
Q: What are the key features of Brown-Sequard syndrome?
Ipsilateral (same side as the lesion):
Weakness below the lesion
Loss of proprioception and vibration sensation
Contralateral (opposite side to the lesion):
Loss of pain and temperature sensation
Q: What are the main uses of carbamazepine?
Epilepsy (particularly partial seizures, first-line treatment)
Trigeminal neuralgia
Bipolar disorder
Q: What is the mechanism of action of carbamazepine?
A: Carbamazepine binds to sodium channels and increases their refractory period, reducing neuronal excitability.
Q: What are common adverse effects of carbamazepine?
Dizziness and ataxia
Drowsiness
Headache
Visual disturbances (especially diplopia)
Stevens-Johnson syndrome
Leucopenia and agranulocytosis
Hyponatraemia due to syndrome of inappropriate ADH secretion
Q: What is a notable feature of carbamazepine’s pharmacokinetics?
A: Carbamazepine exhibits autoinduction, meaning that over time, the drug induces its own metabolism, potentially leading to a return of seizures after 3-4 weeks of treatment.
Q: What effect does carbamazepine have on the P450 enzyme system?
A: Carbamazepine is a P450 enzyme inducer, which can affect the metabolism of other drugs.
Q: What is cataplexy?
A: Cataplexy is the sudden and transient loss of muscular tone triggered by strong emotions, such as laughter or fear.
Q: What percentage of patients with narcolepsy experience cataplexy?
A: Around two-thirds of patients with narcolepsy experience cataplexy.
Q: What are the common features of cataplexy?
A: Features range from buckling knees to complete collapse.
Q: What are the signs of unilateral cerebellar lesions?
A: Unilateral cerebellar lesions cause ipsilateral signs.
Q: What is the mnemonic for symptoms of cerebellar disease?
The mnemonic is DANISH:
D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear ‘Drunk’
A - Ataxia (limb, truncal)
N - Nystagmus (horizontal = ipsilateral hemisphere)
I - Intention tremor
S - Slurred staccato speech, Scanning dysarthria
H - Hypotonia
Q: What are some causes of cerebellar syndrome?
Friedreich’s ataxia, ataxic telangiectasia
Neoplastic (e.g., cerebellar hemangioma)
Stroke
Alcohol
Multiple sclerosis
Hypothyroidism
Drugs (e.g., phenytoin, lead poisoning)
Paraneoplastic (e.g., secondary to lung cancer)
Q: What is cerebral perfusion pressure (CPP)?
A: CPP is the net pressure gradient causing blood flow to the brain, and is tightly autoregulated to maximize cerebral perfusion.
Q: How is cerebral perfusion pressure (CPP) calculated?
CPP = Mean Arterial Pressure (MAP) - Intracranial Pressure (ICP)
Q: What happens if cerebral perfusion pressure (CPP) rises or falls?
A sharp rise in CPP may result in a rising intracranial pressure (ICP).
A fall in CPP may result in cerebral ischemia.
Q: How is CPP managed following trauma?
A: After trauma, CPP must be carefully controlled, which may require invasive monitoring of intracranial pressure (ICP) and mean arterial pressure (MAP).
Q: What is Charcot-Marie-Tooth disease?
A: Charcot-Marie-Tooth disease is the most common hereditary peripheral neuropathy, primarily resulting in motor loss.
Q: What is the management approach for Charcot-Marie-Tooth disease?
A: There is no cure for Charcot-Marie-Tooth disease. Management focuses on physical and occupational therapy.
Q: What are some common features of Charcot-Marie-Tooth disease?
History of frequently sprained ankles
Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity
Q: What is a cluster headache?
A: A cluster headache is an extremely painful headache that occurs in clusters, often once a year, and is more common in men and smokers.
Q: What is the typical pain pattern in cluster headaches?
A: Intense sharp, stabbing pain around one eye, occurring once or twice a day, with each episode lasting 15 minutes to 2 hours.
Q: What are common accompanying features of cluster headaches?
Redness and lacrimation
Lid swelling
Nasal stuffiness
Miosis and ptosis in a minority of cases
Restlessness and agitation during an attack
Q: What is the recommended imaging investigation for cluster headaches?
A: MRI with gadolinium contrast is the investigation of choice, although underlying brain lesions are sometimes found even if the symptoms are typical.
Q: What is the management of acute cluster headaches?
100% oxygen (80% response rate within 15 minutes)
Subcutaneous triptan (75% response rate within 15 minutes)
Q: What is the recommended prophylactic treatment for cluster headaches?
A: Verapamil is the drug of choice, with some evidence supporting a tapering dose of prednisolone.
Q: When should a patient with cluster headaches be referred to a neurologist?
A: Specialist advice should be sought if a patient develops cluster headaches, as specific treatments may be required, such as indomethacin for paroxysmal hemicrania.
Q: Where does the common peroneal nerve originate?
A: The common peroneal nerve is a branch of the sciatic nerve and divides at the neck of the fibula.
Q: What is the most characteristic feature of a common peroneal nerve lesion?
A: Foot drop.
Q: What are the motor deficits associated with a common peroneal nerve lesion?
Weakness of foot dorsiflexion
Weakness of foot eversion
Weakness of the extensor hallucis longus muscle
Q: What sensory loss occurs with a common peroneal nerve lesion?
A: Sensory loss over the dorsum of the foot and the lower lateral part of the leg.
Q: What physical sign is associated with a common peroneal nerve lesion?
A: Wasting of the anterior tibial and peroneal muscles.
Q: What is the function of cranial nerve I (Olfactory)?
A: Smell.
Q: What is the function of cranial nerve II (Optic)?
A: Sight.
Q: What is the function of cranial nerve III (Oculomotor)?
A: Eye movement (medial rectus, inferior oblique, superior rectus, inferior rectus), pupil constriction, accommodation, eyelid opening.
Q: What is the clinical presentation of a lesion in cranial nerve III (Oculomotor)?
A: Ptosis, ‘down and out’ eye, dilated, fixed pupil.
Q: What is the function of cranial nerve IV (Trochlear)?
A: Eye movement (superior oblique).
Q: What is the clinical presentation of a lesion in cranial nerve IV (Trochlear)?
A: Defective downward gaze, vertical diplopia.
Q: What is the function of cranial nerve V (Trigeminal)?
A: Facial sensation, mastication.
Q: What are the potential clinical signs of a lesion in cranial nerve V (Trigeminal)?
A: Trigeminal neuralgia, loss of corneal reflex (afferent), loss of facial sensation, paralysis of mastication muscles, deviation of jaw to the weak side.
Q: What is the function of cranial nerve VI (Abducens)?
A: Eye movement (lateral rectus).
Q: What is the clinical presentation of a lesion in cranial nerve VI (Abducens)?
A: Defective abduction, horizontal diplopia.
Q: What is the function of cranial nerve VII (Facial)?
A: Facial movement, taste (anterior 2/3 of the tongue), lacrimation, salivation.
Q: What are the potential clinical signs of a lesion in cranial nerve VII (Facial)?
A: Flaccid paralysis of upper and lower face, loss of corneal reflex (efferent), loss of taste, hyperacusis.
Q: What is the function of cranial nerve VIII (Vestibulocochlear)?
A: Hearing, balance.
Q: What are the potential clinical signs of a lesion in cranial nerve VIII (Vestibulocochlear)?
A: Hearing loss, vertigo, nystagmus, acoustic neuromas.
Q: What is the function of cranial nerve IX (Glossopharyngeal)?
A: Taste (posterior 1/3 of the tongue), salivation, swallowing, mediates input from the carotid body and sinus.
Q: What are the potential clinical signs of a lesion in cranial nerve IX (Glossopharyngeal)?
A: Hypersensitive carotid sinus reflex, loss of gag reflex (afferent).
Q: What is the function of cranial nerve X (Vagus)?
A: Phonation, swallowing, innervates viscera.
Q: What are the potential clinical signs of a lesion in cranial nerve X (Vagus)?
A: Uvula deviates away from the site of the lesion, loss of gag reflex (efferent).
Q: What is the function of cranial nerve XI (Accessory)?
A: Head and shoulder movement.
Q: What are the potential clinical signs of a lesion in cranial nerve XI (Accessory)?
A: Weakness turning the head to the contralateral side.
Q: What is the function of cranial nerve XII (Hypoglossal)?
A: Tongue movement.
Q: What is the clinical presentation of a lesion in cranial nerve XII (Hypoglossal)?
A: Tongue deviates towards the side of the lesion.
Q: What is the cause of Creutzfeldt-Jakob disease (CJD)?
A: CJD is caused by prion proteins that induce the formation of amyloid folds, resulting in tightly packed beta-pleated sheets resistant to proteases.
Q: What are the key features of Creutzfeldt-Jakob disease (CJD)?
Rapid onset dementia
Myoclonus
Q: What are the typical findings in investigations for Creutzfeldt-Jakob disease (CJD)?
CSF: usually normal
EEG: biphasic, high amplitude sharp waves (only in sporadic CJD)
MRI: hyperintense signals in the basal ganglia and thalamus
Q: What percentage of Creutzfeldt-Jakob disease (CJD) cases are sporadic?
A: 85% of cases are sporadic.
Q: What is the average age of onset for sporadic Creutzfeldt-Jakob disease (CJD)?
A: The mean age of onset is 65 years.
Q: What are the features of new variant Creutzfeldt-Jakob disease (vCJD)?
Affects younger patients (average age of onset = 25 years)
Psychological symptoms such as anxiety, withdrawal, and dysphonia
Prion protein encoded on chromosome 20
Methionine homozygosity at codon 129 of the prion protein is a risk factor
Q: What is the median survival for patients with new variant Creutzfeldt-Jakob disease (vCJD)?
A: The median survival is 13 months.
Q: What are other prion diseases related to Creutzfeldt-Jakob disease (CJD)?
Kuru
Fatal familial insomnia
Gerstmann Straussler-Scheinker disease
Q: What are the main risk factors for developing degenerative cervical myelopathy (DCM)?
Smoking (due to its effects on intervertebral discs)
Genetics
Occupations with high axial loading
Q: What are the common symptoms of degenerative cervical myelopathy (DCM)?
Pain (neck, upper or lower limbs)
Loss of motor function (e.g., difficulty with digital dexterity, arm or leg weakness)
Loss of sensory function (e.g., numbness)
Loss of autonomic function (e.g., urinary or fecal incontinence, impotence)
Hoffman’s sign (positive reflex test for cervical myelopathy)
Q: What is Hoffman’s sign and what does it indicate?
Hoffman’s sign is a reflex test where a flick of the finger on a patient’s hand causes twitching of the other fingers, indicating cervical myelopathy.
Q: How is degenerative cervical myelopathy (DCM) diagnosed?
An MRI of the cervical spine is the gold standard test, which can reveal disc degeneration, ligament hypertrophy, and cord signal changes.
Q: Why is early diagnosis and treatment crucial in degenerative cervical myelopathy (DCM)?
Early treatment (within 6 months) offers the best chance for full recovery, as any existing spinal cord damage may be permanent. Delayed diagnosis can result in irreversible damage.
Q: What is the only effective treatment for degenerative cervical myelopathy (DCM)?
Decompressive surgery is the only effective treatment, which can prevent disease progression. Close observation is an option for stable, mild disease.
Q: Why should physiotherapy be initiated only by specialist services in degenerative cervical myelopathy (DCM)?
Physiotherapy should be initiated by specialist services to prevent further spinal cord damage, as manipulation can worsen the condition.
Q: What is the typical delay in diagnosis for patients with degenerative cervical myelopathy (DCM)?
Patients often experience an average of over 5 appointments and more than 2 years before being diagnosed with DCM.
Q: Which drug is known to cause peripheral neuropathy and is commonly used in the treatment of arrhythmias?
Amiodarone
Q: Which drug, used primarily for the treatment of tuberculosis, can cause peripheral neuropathy?
Isoniazid
Q: Which chemotherapy drug, used in the treatment of various cancers, is associated with peripheral neuropathy?
Vincristine
Q: Which antibiotic, often used for urinary tract infections, can cause peripheral neuropathy?
Nitrofurantoin
Q: Which antibiotic, used for bacterial infections, can cause peripheral neuropathy as a side effect?
Metronidazole
Q: How long must a patient with an isolated first unprovoked seizure wait before they can resume driving if brain imaging and EEG are normal?
6 months
Q: For patients with established epilepsy or multiple unprovoked seizures, how long must they be seizure-free before being eligible to drive again?
12 months
Q: When withdrawing anti-epilepsy medication, how long should a patient wait after the last dose before resuming driving?
6 months
Q: What is the driving restriction for a patient who has experienced a single unexplained episode of syncope?
6 months off
Q: How long should a patient who has had two or more episodes of syncope refrain from driving?
12 months
Q: After a stroke or TIA, how long must a patient wait before driving if there is no residual neurological deficit?
1 month
Q: What is the required driving restriction for multiple TIAs occurring over a short period?
3 months off driving, and the DVLA must be informed
Q: After craniotomy for meningioma, how long must a patient refrain from driving?
1 year
Q: What is the inheritance pattern of dystrophinopathies?
X-linked recessive
Q: What is the role of dystrophin in muscle cells?
Dystrophin connects the muscle membrane to actin, a part of the muscle cytoskeleton
Q: What is the typical age of onset for Duchenne muscular dystrophy?
Progressive proximal muscle weakness begins around 5 years of age
Q: What is a characteristic sign of Duchenne muscular dystrophy?
Calf pseudohypertrophy
Q: What is Gower’s sign?
A child uses their arms to stand up from a squatted position, seen in Duchenne muscular dystrophy
Q: What percentage of Duchenne muscular dystrophy patients experience intellectual impairment?
30%
Q: At what age does Becker muscular dystrophy typically develop?
After the age of 10 years
Q: Is intellectual impairment common in Becker muscular dystrophy?
No, it is much less common compared to Duchenne muscular dystrophy
Q: What are the general EMG characteristics for neuropathy?
Increased action potential duration
Increased action potential amplitude
Q: What are the general EMG characteristics for myopathy?
Decreased action potential duration
Decreased action potential amplitude
Q: What is the characteristic EMG finding for myasthenia gravis?
Diminished response to repetitive stimulation
Q: What is the characteristic EMG finding for Lambert-Eaton syndrome?
Incremental response to repetitive stimulation
Q: What is the characteristic EMG finding for myotonic syndromes?
Extended series of repetitive discharges lasting up to 30 seconds
Q: What are the common features of encephalitis?
Fever
Headache
Psychiatric symptoms
Seizures
Vomiting
Focal features (e.g., aphasia)
Q: What is the pathophysiology behind most cases of encephalitis in adults?
HSV-1 (Herpes Simplex Virus-1) is responsible for 95% of cases in adults, typically affecting the temporal and inferior frontal lobes.
Q: What are the typical cerebrospinal fluid findings in encephalitis?
Lymphocytosis
Elevated protein
PCR for HSV, VZV, and enteroviruses
Q: What neuroimaging findings are seen in encephalitis?
Medial temporal and inferior frontal changes (e.g., petechial hemorrhages)
MRI is better than CT
Normal in one-third of patients
Q: What is the management for suspected encephalitis?
Intravenous aciclovir should be started in all suspected cases.
Q: What are the key features of infantile spasms (West’s syndrome)?
Brief spasms beginning in the first few months of life
Flexion of head, trunk, limbs → extension of arms (Salaam attack)
Lasts 1-2 seconds, repeat up to 50 times
Progressive mental handicap
EEG: Hypsarrhythmia
Often secondary to neurological abnormalities (e.g., tuberous sclerosis, encephalitis, birth asphyxia), or may be idiopathic
Treatment: Vigabatrin and steroids
Prognosis: Poor
Q: What are the features of typical (petit mal) absence seizures?
Onset: 4-8 years
Duration: Few seconds to 30 seconds
No warning, quick recovery, often multiple episodes per day
EEG: 3Hz generalized, symmetrical
Treatment: Sodium valproate, ethosuximide
Prognosis: Good (90-95% become seizure-free in adolescence)
Q: What are the features of Lennox-Gastaut syndrome?
Onset: 1-5 years
May develop from infantile spasms
Features: Atypical absences, falls, jerks
90% have moderate to severe mental handicap
EEG: Slow spike waves
Treatment: Ketogenic diet may help
Q: What are the key features of benign rolandic epilepsy?
Most common in childhood, more common in males
Features: Unilateral facial paraesthesia, usually upon waking up
Q: What are the features of juvenile myoclonic epilepsy (Janz syndrome)?
Onset: Teenage years, more common in girls
Features: Infrequent generalized seizures, often in the morning or after sleep deprivation
Daytime absences
Sudden, shock-like myoclonic seizures (may develop before generalized seizures)
Treatment: Usually responds well to sodium valproate
Q: What are some conditions associated with epilepsy?
Cerebral palsy (30% have epilepsy)
Tuberous sclerosis
Mitochondrial diseases
Q: What are common causes of recurrent seizures other than epilepsy?
Febrile convulsions: Typically in children 6 months to 5 years, triggered by a rapid rise in temperature during a viral infection. Seizures are brief and generalized.
Alcohol withdrawal seizures: Occur in patients with a history of alcohol excess who suddenly stop drinking, typically peaking at 36 hours after cessation.
Psychogenic non-epileptic seizures: Seizures without electrical discharges, often in patients with mental health issues or personality disorders.
Q: What are the key features in the classification of seizures?
Where seizures begin in the brain
Level of awareness during a seizure
Other features of seizures
Q: What are the types of focal seizures?
Focal aware seizures: Seizures that start in one area of the brain with preserved awareness.
Focal impaired awareness seizures: Seizures that start in one area of the brain with impaired awareness.
Focal seizures with other features: Can include motor (e.g., Jacksonian march), non-motor (e.g., déjà vu), or other features like aura.
Q: What are generalised seizures and their subtypes?
Generalized seizures engage networks on both sides of the brain, with consciousness lost immediately. Subtypes include:
Tonic-clonic (grand mal)
Tonic
Clonic
Typical absence (petit mal)
Myoclonic
Atonic
Q: What is a focal to bilateral seizure?
A seizure that starts in one area of the brain and spreads to both lobes, previously termed secondary generalized seizures.
Q: What are some special forms of epilepsy in children?
Infantile spasms (West’s syndrome): Brief spasms, often with hypsarrhythmia on EEG.
Lennox-Gastaut syndrome: A severe form of epilepsy with slow spike waves on EEG, often associated with mental handicap.
Benign rolandic epilepsy: Unilateral facial paraesthesia, seizures occur at night.
Juvenile myoclonic epilepsy (Janz syndrome): Myoclonic seizures often triggered by sleep deprivation, responds well to sodium valproate.
Q: What are some signs and symptoms following a generalized seizure?
Tongue biting
Incontinence of urine
Postictal drowsiness and fatigue for about 15 minutes
Q: What investigations are typically done after a first seizure?
EEG (electroencephalogram)
Neuroimaging (usually MRI)
Q: When should antiepileptics be started for epilepsy?
Antiepileptics are generally started after a second seizure.
Q: What are some common antiepileptic drugs and their adverse effects?
Sodium valproate: Increases GABA activity, used for generalized seizures; adverse effects include weight gain, alopecia, ataxia, and teratogenicity.
Carbamazepine: Sodium channel blocker, used for focal seizures; adverse effects include dizziness, leucopenia, and diplopia.
Lamotrigine: Sodium channel blocker, used for generalized and focal seizures; adverse effects include Stevens-Johnson syndrome.
Phenytoin: Sodium channel blocker, used for focal seizures; adverse effects include gingival hyperplasia, hirsutism, and peripheral neuropathy.
Q: How are seizures managed acutely?
Seizures often stop spontaneously, but if they last longer than 5-10 minutes, medications like benzodiazepines (e.g., diazepam) may be used. In cases of status epilepticus, further treatment with benzodiazepines, antiepileptic infusions, or general anaesthesia may be needed.
Q: What are focal seizures and how are they classified?
Focal seizures start in a specific area of the brain on one side. They can be classified based on the level of awareness and other features:
Focal aware seizures (previously ‘simple partial’)
Focal impaired awareness seizures (previously ‘complex partial’)
Awareness unknown
Further classification includes:
Motor features (e.g., Jacksonian march)
Non-motor features (e.g., déjà vu, jamais vu)
Other features (e.g., aura)
Q: What are generalized seizures and their subtypes?
Generalized seizures engage both sides of the brain at onset, causing immediate loss of consciousness. They can be further subdivided into:
Motor generalized seizures (e.g., tonic-clonic)
Non-motor generalized seizures (e.g., absence) Specific types include:
Tonic-clonic (grand mal)
Tonic
Clonic
Typical absence (petit mal)
Atonic
Q: What are typical features of focal seizures originating in the temporal lobe?
Seizures may occur with or without impairment of consciousness or awareness
Aura: Most patients experience a rising epigastric sensation, often accompanied by psychic phenomena (e.g., déjà vu, jamais vu)
Less commonly, hallucinations (auditory, gustatory, olfactory)
Seizures typically last around one minute
Automatisms: Lip smacking, grabbing, plucking
Q: What are the typical features of focal seizures originating in the frontal lobe (motor)?
Head/leg movements
Posturing
Post-ictal weakness
Jacksonian march (sequential motor involvement from one body part to another)
Q: What are the typical features of focal seizures originating in the parietal lobe (sensory)?
Paraesthesia (tingling or numbness)
Q: What are the typical features of focal seizures originating in the occipital lobe (visual)?
Floaters or flashes (visual disturbances)
Q: When should antiepileptic drugs (AEDs) be started according to NICE guidelines?
A: AEDs should be started after the first seizure if:
The patient has a neurological deficit
Brain imaging shows a structural abnormality
The EEG shows unequivocal epileptic activity
The patient or family considers the risk of a further seizure unacceptable
Q: What is the first-line treatment for generalised tonic-clonic seizures in males?
A: Sodium valproate
Q: What is the first-line treatment for generalised tonic-clonic seizures in females?
A: Lamotrigine or Levetiracetam
Girls under 10 years old or women unable to have children may be offered sodium valproate first-line.
Q: What is the first-line treatment for focal seizures?
A: Lamotrigine or Levetiracetam
Q: What is the first-line treatment for absence seizures (Petit mal)?
A: Ethosuximide
Second-line:
Males: Sodium valproate
Females: Lamotrigine or Levetiracetam
Carbamazepine may exacerbate absence seizures.
Q: What is the first-line treatment for myoclonic seizures in males?
A: Sodium valproate
Q: What is the first-line treatment for myoclonic seizures in females?
A: Levetiracetam
Q: What is the first-line treatment for tonic or atonic seizures in males?
A: Sodium valproate
Q: What is the first-line treatment for tonic or atonic seizures in females?
A: Lamotrigine
Q: What is the inheritance pattern of essential tremor?
A: Autosomal dominant
Q: What is the most common feature of essential tremor?
A: Postural tremor, which is worse when the arms are outstretched.
Q: What can improve the tremor in essential tremor?
A: Alcohol and rest can improve the tremor.
Q: What is the most common cause of titubation (head tremor)?
A: Essential tremor is the most common cause of titubation.
Q: What is the first-line treatment for essential tremor?
A: Propranolol is the first-line treatment.
Q: What is another treatment option for essential tremor if propranolol is ineffective?
A: Primidone is sometimes used if propranolol is ineffective.
Q: What is an extradural haematoma?
A: A collection of blood between the skull and the dura.
Q: What is the most common cause of an extradural haematoma?
A: Trauma, most typically from low-impact injuries (e.g., a blow to the head or a fall).
Q: Where is an extradural haematoma most commonly located?
A: In the temporal region, especially at the pterion overlying the middle meningeal artery.
Q: What is the classic presentation of an extradural haematoma?
A: Loss of consciousness, followed by a brief recovery (lucid interval), then another loss of consciousness due to expanding haematoma and brain herniation.
Q: What is the cause of a fixed and dilated pupil in extradural haematoma?
A: Compression of the parasympathetic fibers of the third cranial nerve due to temporal lobe uncus herniation around the tentorium cerebelli.
Q: How does an extradural haematoma appear on imaging?
A: A biconvex (lentiform) hyperdense collection around the surface of the brain, limited by the suture lines of the skull.
Q: What is the treatment for an extradural haematoma?
A: Craniotomy and evacuation of the haematoma is the definitive treatment. For patients with no neurological deficit, clinical and radiological observation may be appropriate.
Q: What is the main function of the facial nerve?
A: It supplies muscles of facial expression, the digastric muscle, and many glandular structures. It also carries taste sensation from the anterior two-thirds of the tongue and parasympathetic fibers to the lacrimal and salivary glands.
Q: What is the mnemonic for the supply of the facial nerve?
A: “Face, ear, taste, tear”
Face: Muscles of facial expression
Ear: Nerve to stapedius
Taste: Anterior two-thirds of the tongue
Tear: Parasympathetic fibers to lacrimal and salivary glands
Q: What are the causes of bilateral facial nerve palsy?
A: Sarcoidosis, Guillain-Barre syndrome, Lyme disease, bilateral acoustic neuromas (neurofibromatosis type 2), and Bell’s palsy (25% of bilateral cases, but only 1% of Bell’s palsy cases).
Q: What are the causes of unilateral facial nerve palsy?
A: Bell’s palsy, Ramsay-Hunt syndrome (herpes zoster), acoustic neuroma, parotid tumors, HIV, multiple sclerosis, diabetes mellitus (lower motor neuron), and stroke (upper motor neuron).
Q: How do you differentiate between upper and lower motor neuron lesions affecting the facial nerve?
A: Upper motor neuron lesion spares the upper face (forehead), while a lower motor neuron lesion affects all facial muscles.
Q: What is the path of the facial nerve?
A: It originates in the pons and passes through the petrous temporal bone into the internal auditory meatus with the vestibulocochlear nerve. It then travels through the facial canal, where it gives off three branches: greater petrosal nerve, nerve to stapedius, and chorda tympani. It exits the skull through the stylomastoid foramen and gives branches to the posterior auricular nerve, posterior belly of digastric muscle, and stylohyoid muscle.
Q: What is foot drop?
A: Foot drop is weakness of the foot dorsiflexors, causing difficulty lifting the foot during walking.
Q: What is the most common cause of foot drop?
A: Common peroneal nerve lesion, typically due to compression at the neck of the fibula.
Q: What are some common causes of common peroneal nerve lesions?
A: Compression from leg crossing, squatting, kneeling, prolonged confinement, recent weight loss, Baker’s cysts, or plaster casts.
Q: What are other possible causes of foot drop besides common peroneal nerve lesions?
A: L5 radiculopathy, sciatic nerve lesion, superficial or deep peroneal nerve lesion, and central nerve lesions (e.g., stroke).
Q: What are the key features of an isolated peroneal neuropathy on examination?
A: Weakness of foot dorsiflexion and eversion, with normal reflexes.
Q: What would weakness of hip abduction suggest on examination?
A: L5 radiculopathy.
Q: When should a patient with foot drop be referred to a specialist?
A: If there are bilateral symptoms, fasciculations, or other abnormal neurological findings (e.g., hyperreflexia).
Q: What is the management for isolated peroneal neuropathy?
A: Conservative management, including avoiding leg crossing, squatting, and kneeling. Symptoms typically improve over 2-3 months.
Q: What does the fourth cranial nerve (trochlear nerve) supply?
A: The fourth cranial nerve supplies the superior oblique muscle.
Q: What is the function of the superior oblique muscle?
A: The superior oblique muscle is responsible for rotating the eye downward and outward, especially when the eye is adducted.
Q: What are the common symptoms of fourth nerve palsy?
A: Vertical diplopia (double vision), subjective tilting of objects (torsional diplopia), and a head tilt.
Q: How does the affected eye appear in a patient with fourth nerve palsy when looking straight ahead?
A: The affected eye appears to deviate upwards and is rotated outwards.
Q: What type of inheritance pattern does Friedreich’s ataxia follow?
A: Friedreich’s ataxia is an autosomal recessive disorder.
Q: What is unique about Friedreich’s ataxia compared to other trinucleotide repeat disorders?
A: It does not demonstrate the phenomenon of anticipation.
Q: What is the typical age of onset for Friedreich’s ataxia?
A: The typical age of onset is between 10 and 15 years old.
Q: What are the most common presenting features of Friedreich’s ataxia?
A: Gait ataxia and kyphoscoliosis.
Q: What are the key neurological features of Friedreich’s ataxia?
A: Absent ankle jerks, extensor plantars, cerebellar ataxia, optic atrophy, and spinocerebellar tract degeneration.
Q: What is the most common cause of death in Friedreich’s ataxia?
A: Hypertrophic obstructive cardiomyopathy (90% of cases).
Q: What other conditions are commonly associated with Friedreich’s ataxia?
A: Diabetes mellitus (10-20%) and high-arched palate.
Q: How does Friedreich’s ataxia compare to ataxic telangiectasia in terms of age of onset?
A: Ataxic telangiectasia tends to present much earlier, often at 1-2 years of age, whereas Friedreich’s ataxia typically presents between 10 and 15 years.
Q: What are the options for motor response in the Glasgow Coma Scale?
6: Obeys commands
5: Localises to pain
4: Withdraws from pain
3: Abnormal flexion to pain (decorticate posture)
2: Extending to pain
1: None
Q: What are the options for verbal response in the Glasgow Coma Scale?
5: Orientated
4: Confused
3: Words
2: Sounds
1: None
Q: What are the options for eye opening in the Glasgow Coma Scale?
4: Spontaneous
3: To speech
2: To pain
1: None
Q: What is the pathogenesis of Guillain-Barré syndrome?
A: Guillain-Barré syndrome is caused by immune-mediated demyelination of the peripheral nervous system. It is often triggered by an infection, such as Campylobacter jejuni. The pathogenesis involves the cross-reaction of antibodies with gangliosides in the peripheral nervous system.
Q: What is the significance of anti-ganglioside antibodies in Guillain-Barré syndrome?
A: Anti-ganglioside antibodies, such as anti-GM1, are found in 25% of Guillain-Barré syndrome patients. These antibodies correlate with clinical features, contributing to the disease process.
Q: What is Miller Fisher syndrome?
A: Miller Fisher syndrome is a variant of Guillain-Barré syndrome, characterized by ophthalmoplegia, areflexia, and ataxia. It usually presents as a descending paralysis (starting from the eyes) rather than the typical ascending paralysis seen in other forms of Guillain-Barré syndrome.
Q: What antibodies are associated with Miller Fisher syndrome?
A: Anti-GQ1b antibodies are present in 90% of cases of Miller Fisher syndrome.
Q: What are the initial symptoms of Guillain-Barré syndrome?
A: Back/leg pain is experienced by around 65% of patients in the initial stages of Guillain-Barré syndrome.
Q: What is the characteristic feature of Guillain-Barré syndrome?
A: The characteristic feature is progressive, symmetrical weakness of all limbs, typically starting in the legs (ascending weakness). Reflexes are reduced or absent.
Q: What are common sensory symptoms in Guillain-Barré syndrome?
A: Sensory symptoms in Guillain-Barré syndrome tend to be mild, such as distal paraesthesia, with very few sensory signs.
Q: What other features may be present in Guillain-Barré syndrome?
History of gastroenteritis (often due to Campylobacter jejuni)
Respiratory muscle weakness
Cranial nerve involvement (e.g., diplopia, bilateral facial nerve palsy)
Oropharyngeal weakness
Autonomic involvement (e.g., urinary retention, diarrhoea)
Q: What are less common findings in Guillain-Barré syndrome?
A: Papilloedema (due to reduced CSF resorption) is a less common finding in Guillain-Barré syndrome.
Q: What findings on lumbar puncture are suggestive of Guillain-Barré syndrome?
A: Lumbar puncture typically shows albuminocytologic dissociation, where there is a rise in protein with a normal white blood cell count in 66% of patients.
Q: What abnormalities might be seen on nerve conduction studies in Guillain-Barré syndrome?
Decreased motor nerve conduction velocity (due to demyelination)
Prolonged distal motor latency
Increased F wave latency
**Q: What are common features of a migraine?
A: Migraine is a recurrent, severe headache that is usually unilateral and throbbing. It may be associated with aura, nausea, and photosensitivity. The headache is aggravated by, or causes avoidance of, routine activities, and patients often feel the need to “go to bed.” In women, it may be associated with menstruation.
**Q: What are the characteristics of a tension headache?
A: Tension headache is recurrent, non-disabling, and bilateral, often described as a “tight-band” sensation. It is not aggravated by routine activities of daily living.
**Q: What are the key features of a cluster headache?
A: Cluster headache typically involves intense pain around one eye, with recurrent attacks lasting 15 minutes to 2 hours. Attacks occur once or twice a day and last for 4-12 weeks. Patients are restless during attacks, and the headache is accompanied by redness, lacrimation, and lid swelling. It is more common in men and smokers.
**Q: What are the characteristics of temporal arteritis?
A: Temporal arteritis typically affects patients over 60 years old and presents with rapid onset unilateral headache (usually < 1 month). It is associated with jaw claudication (65%), a tender, palpable temporal artery, and a raised ESR.
**Q: What defines medication overuse headache?
A: Medication overuse headache occurs when headaches are present for 15 days or more per month and worsen while taking regular symptomatic medication. Patients using opioids and triptans are at the highest risk, and psychiatric comorbidity may also be present.
**Q: What are some possible causes of acute single-episode headache?
Meningitis
Encephalitis
Subarachnoid hemorrhage
Head injury
Sinusitis
Glaucoma (acute closed-angle)
Tropical illness (e.g., Malaria)
**Q: What are some causes of chronic headache?
Chronically raised intracranial pressure (ICP)
Paget’s disease
Psychological causes
**Q: What is the recommended management for trigeminal autonomic cephalgia (including cluster headaches)?
A: It is recommended to refer patients with trigeminal autonomic cephalgia (such as cluster headache, paroxysmal hemicrania, and SUNCT) for specialist assessment. Specific treatments, such as indomethacin for paroxysmal hemicrania, may be required.
**Q: What are some red flags for a headache in a patient with compromised immunity?
A: Red flags for compromised immunity include a history of HIV or immunosuppressive drug use, which could increase the risk of infections like meningitis or brain abscesses.
**Q: What headache red flag is associated with patients under 20 years of age and a history of malignancy?
A: A headache in patients under 20 years old with a history of malignancy, particularly one known to metastasize to the brain, is a red flag that warrants further investigation.
**Q: What headache feature suggests a thunderclap headache?
A: A thunderclap headache is characterized by sudden-onset headache that reaches maximum intensity within 5 minutes, and it can be indicative of serious conditions like subarachnoid hemorrhage.
**Q: What does vomiting without other obvious cause in a headache patient suggest?
A: Vomiting without an obvious cause may indicate increased intracranial pressure or conditions such as a brain tumor or hemorrhage.
**Q: When should fever accompanying a headache raise concern?
A: Worsening headache with fever can be a red flag for conditions like meningitis or encephalitis and should be evaluated urgently.
**Q: What should be considered in a patient with new-onset neurological deficits or cognitive dysfunction?
A: New-onset neurological deficits or cognitive dysfunction with headache could indicate serious conditions like a brain tumor, stroke, or brain abscess.
**Q: What features should prompt concern in a patient with a change in personality?
A: A change in personality associated with headache could indicate a space-occupying lesion, such as a brain tumor or abscess.
**Q: What headache presentation could suggest a brain injury?
A: A recent head trauma, particularly within the past 3 months, along with headache, warrants investigation for post-concussion syndrome, hematoma, or other traumatic brain injury-related conditions.
**Q: What is a red flag if a headache is triggered by certain activities?
A: Headache triggered by coughing, Valsalva maneuver (bearing down), sneezing, or exercise can suggest a structural brain issue, such as a tumor or aneurysm, and needs further evaluation.
**Q: What is the significance of orthostatic headache (headache that changes with posture)?
A: Orthostatic headache (worsening or improving with posture) may indicate conditions like a CSF leak or increased intracranial pressure and should be assessed promptly.
**Q: What are symptoms of giant cell arteritis or acute narrow-angle glaucoma to watch for?
A: Symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma include scalp tenderness, jaw claudication, visual disturbances, and eye pain, which require urgent medical attention.
**Q: What does a substantial change in headache characteristics indicate?
A: A substantial change in the pattern, intensity, or frequency of a patient’s headache could indicate a secondary cause such as a brain tumor, intracranial hemorrhage, or other serious pathology.
**Q: What is the common cause of Herpes Simplex Encephalitis (HSE) in adults?
A: HSV-1 is responsible for 95% of cases of Herpes Simplex Encephalitis in adults.
**Q: What is the typical presentation of Herpes Simplex Encephalitis?
A: Typical features include fever, headache, psychiatric symptoms, seizures, vomiting, and focal neurological signs such as aphasia.
**Q: Which part of the brain is most commonly affected in Herpes Simplex Encephalitis?
A: The temporal lobes are most commonly affected in Herpes Simplex Encephalitis.
**Q: How are peripheral lesions like cold sores related to Herpes Simplex Encephalitis?
A: Peripheral lesions, such as cold sores, are not related to the presence of Herpes Simplex Encephalitis.
**Q: What CSF findings are typical in Herpes Simplex Encephalitis?
A: CSF findings include lymphocytosis, elevated protein, and a positive PCR for HSV.
**Q: What is the imaging modality of choice to diagnose Herpes Simplex Encephalitis?
A: MRI is better than CT for detecting Herpes Simplex Encephalitis. CT may show medial temporal and inferior frontal changes, while MRI reveals hyperintensity in the affected white matter and cortex.
**Q: What is the typical EEG pattern seen in Herpes Simplex Encephalitis?
A: The EEG pattern typically shows lateralised periodic discharges at 2 Hz in Herpes Simplex Encephalitis.
**Q: What is the first-line treatment for Herpes Simplex Encephalitis?
A: The first-line treatment is intravenous aciclovir.
**Q: What is the newer term for Charcot-Marie-Tooth disease?
A: The newer term for Charcot-Marie-Tooth disease is Hereditary Sensorimotor Neuropathy (HSMN).
**Q: What are the two main types of Hereditary Sensorimotor Neuropathy (HSMN)?
HSMN type I (demyelinating pathology)
HSMN type II (axonal pathology)
**Q: What is the genetic inheritance pattern of HSMN type I?
A: HSMN type I follows an autosomal dominant inheritance pattern.
**Q: At what age do features of HSMN type I typically start to appear?
A: Features of HSMN type I often start at puberty.
**Q: What is the predominant symptom in HSMN type I?
A: The predominant symptom in HSMN type I is motor symptoms, including distal muscle wasting and weakness.
**Q: What are common physical findings in HSMN type I?
Distal muscle wasting
Pes cavus
Clawed toes
Foot drop
Leg weakness
**Q: What is the main clinical feature at the onset of HSMN type I?
A: The main clinical features at the onset of HSMN type I include foot drop and leg weakness.
**Q: What is the genetic inheritance pattern of Huntington’s disease?
A: Huntington’s disease follows an autosomal dominant inheritance pattern.
**Q: What genetic abnormality causes Huntington’s disease?
A: Huntington’s disease is caused by a trinucleotide repeat expansion of CAG in the huntingtin gene on chromosome 4.
**Q: What is the phenomenon of anticipation in Huntington’s disease?
A: Anticipation refers to the phenomenon where Huntington’s disease presents at an earlier age in successive generations due to the trinucleotide repeat disorder.
**Q: What type of neurons are affected in Huntington’s disease?
A: Huntington’s disease results in the degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia.
**Q: At what age do symptoms of Huntington’s disease typically develop?
A: Symptoms of Huntington’s disease typically develop after 35 years of age.
**Q: What are the typical motor symptoms of Huntington’s disease?
A: The typical motor symptoms of Huntington’s disease include chorea and dystonia.
**Q: What are common psychiatric and cognitive features of Huntington’s disease?
Personality changes (e.g., irritability, apathy, depression)
Intellectual impairment
Saccadic eye movements
**Q: What is idiopathic intracranial hypertension (IIH), and what is its other name?
A: Idiopathic intracranial hypertension (IIH) is also known as pseudotumour cerebri and was formerly called benign intracranial hypertension.
**Q: What is the typical demographic affected by idiopathic intracranial hypertension?
A: Idiopathic intracranial hypertension is classically seen in young, overweight females.
**Q: What are the key risk factors for idiopathic intracranial hypertension?
Obesity
Female sex
Pregnancy
Certain medications (e.g., combined oral contraceptive pill, steroids, tetracyclines, retinoids, lithium).
**Q: What are the main features of idiopathic intracranial hypertension?
Headache
Blurred vision
Papilloedema (usually present)
Enlarged blind spot
Sixth nerve palsy may be present.
**Q: What are the first-line management strategies for idiopathic intracranial hypertension?
Weight loss (via diet and exercise)
Carbonic anhydrase inhibitors (e.g., acetazolamide)
Topiramate (inhibits carbonic anhydrase and promotes weight loss).
**Q: What surgical options are available for idiopathic intracranial hypertension?
Optic nerve sheath decompression and fenestration (to prevent optic nerve damage)
Lumboperitoneal or ventriculoperitoneal shunts (to reduce intracranial pressure).
**Q: What is internuclear ophthalmoplegia (INO), and what causes it?
A: Internuclear ophthalmoplegia (INO) is a cause of horizontal disconjugate eye movement due to a lesion in the medial longitudinal fasciculus (MLF).
**Q: What is the role of the medial longitudinal fasciculus (MLF) in eye movement?
A: The MLF controls horizontal eye movements by interconnecting the IIIrd, IVth, and VIth cranial nuclei, which are located in the paramedian area of the midbrain and pons.
**Q: What are the key features of internuclear ophthalmoplegia (INO)?
Impaired adduction of the eye on the same side as the lesion
Horizontal nystagmus of the abducting eye on the contralateral side.
**Q: What are the common causes of internuclear ophthalmoplegia?
Multiple sclerosis
Vascular disease.
**Q: What is intracranial venous thrombosis, and what does it cause?
A: Intracranial venous thrombosis can cause cerebral infarction, but it is much less common than arterial causes.
**Q: What is the most common type of intracranial venous thrombosis?
A: Around 50% of patients have isolated sagittal sinus thrombosis. The remainder have coexistent lateral sinus and cavernous sinus thromboses.
**Q: What are the general features of intracranial venous thrombosis?
Headache (which may be sudden onset)
Nausea & vomiting
Reduced consciousness.
**Q: What is the gold standard investigation for intracranial venous thrombosis?
A: MRI venography is the gold standard. CT venography is an alternative, but a non-contrast CT head is normal in about 70% of patients. D-dimer levels may also be elevated.
**Q: What is the initial management for intracranial venous thrombosis?
A: The typical management is anticoagulation:
Low molecular weight heparin is used acutely
Warfarin is typically used for long-term anticoagulation.
**Q: What are the features of sagittal sinus thrombosis?
Seizures and hemiplegia
Parasagittal biparietal or bifrontal haemorrhagic infarctions may be seen
‘Empty delta sign’ on venography.
**Q: What are the features of cavernous sinus thrombosis?
Periorbital erythema and oedema
Ophthalmoplegia (6th nerve damage occurs before 3rd & 4th)
Trigeminal nerve involvement, causing hyperaesthesia of the upper face and eye pain
Central retinal vein thrombosis.
**Q: What are the features of lateral sinus thrombosis?
A: Features include 6th and 7th cranial nerve palsies.
**Q: What is Lambert-Eaton syndrome, and what is its association with cancer?
A: Lambert-Eaton syndrome is often seen in association with small cell lung cancer and, to a lesser extent, breast and ovarian cancer. It can also occur independently as an autoimmune disorder.
**Q: What causes Lambert-Eaton myasthenic syndrome?
A: Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channels in the peripheral nervous system.
**Q: What is the typical presentation of Lambert-Eaton syndrome?
repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis)
Limb-girdle weakness, often starting in the lower limbs
Hyporeflexia
Autonomic symptoms: dry mouth, impotence, and difficulty micturating
Unlike in myasthenia gravis, ophthalmoplegia and ptosis are not commonly seen.
**Q: What is the response to repetitive electrical stimulation in Lambert-Eaton syndrome?
A: EMG shows an incremental response to repetitive electrical stimulation.
**Q: How is Lambert-Eaton syndrome managed?
Treatment of the underlying cancer
Immunosuppression, for example with prednisolone and/or azathioprine
3,4-diaminopyridine is being trialled, which increases acetylcholine release by blocking potassium channel efflux at the nerve terminal
Intravenous immunoglobulin therapy and plasma exchange may be beneficial.
**Q: What is the primary use of Lamotrigine?
A: Lamotrigine is used as a second-line antiepileptic for a variety of generalized and partial seizures.
**Q: What is the mechanism of action of Lamotrigine?
A: Lamotrigine acts as a sodium channel blocker, inhibiting the spread of seizure activity.
**Q: What is a serious adverse effect of Lamotrigine?
A: A serious adverse effect of Lamotrigine is Stevens-Johnson syndrome, a life-threatening skin condition.
**Q: What is the cause of Lateral Medullary Syndrome (Wallenberg’s Syndrome)?
A: Lateral Medullary Syndrome is caused by occlusion of the posterior inferior cerebellar artery.
**Q: What cerebellar features are associated with Lateral Medullary Syndrome?
A: Lateral Medullary Syndrome presents with ataxia and nystagmus as cerebellar features.
**Q: What are the brainstem features of Lateral Medullary Syndrome?
A: Ipsilateral features include dysphagia, facial numbness, and cranial nerve palsy (e.g., Horner’s syndrome).
Contralateral features include limb sensory loss.
**Q: Why is levodopa usually combined with a decarboxylase inhibitor (e.g., carbidopa or benserazide)?
A: Levodopa is combined with a decarboxylase inhibitor to prevent the peripheral metabolism of L-dopa to dopamine, ensuring more L-dopa reaches the brain.
**Q: What is a common issue with levodopa treatment over time?
A: The effectiveness of levodopa typically reduces over time, usually by around 2 years.
**Q: In which condition is levodopa not useful?
A: Levodopa is not useful in neuroleptic-induced parkinsonism.
**Q: What are the common adverse effects of levodopa?
Dyskinesia
‘On-off’ effect
Postural hypotension
Cardiac arrhythmias
Nausea & vomiting
Psychosis
Reddish discoloration of urine upon standing
**Q: How is the median nerve formed and what are its roots?
A: The median nerve is formed by the union of a lateral root (C5, C6, C7) and a medial root (C8, T1) from the brachial plexus.
**Q: What sensory areas does the median nerve innervate?
A: The median nerve provides sensory innervation to the palmar aspect of the thumb and lateral 2 ½ fingers and the distal regions on the dorsal aspect of the same fingers.
**Q: What are the effects of damage to the median nerve at the wrist (e.g., carpal tunnel syndrome)?
Paralysis and wasting of the thenar eminence muscles
Opponens pollicis paralysis (leading to ape hand deformity)
Sensory loss to the palmar aspect of the lateral 2 ½ fingers.
**Q: What happens with median nerve damage at the elbow?
Inability to pronate the forearm
Weak wrist flexion
Ulnar deviation of the wrist.
**Q: What does damage to the anterior interosseous nerve (a branch of the median nerve) result in?
Loss of pronation of the forearm
Weakness of the long flexors of the thumb and index finger.
**Q: What is medication overuse headache?
A: Medication overuse headache is one of the most common causes of chronic daily headache, affecting up to 1 in 50 people. It occurs when headaches are present for 15 days or more per month and develop or worsen with the use of regular symptomatic medication.
**Q: Which medications are most commonly associated with medication overuse headache?
A: Opioids and triptans are the most common medications associated with medication overuse headache.
**Q: What is the management approach for medication overuse headache according to the 2008 SIGN guidelines?
Simple analgesics and triptans should be withdrawn abruptly (which may initially worsen headaches).
Opioid analgesics should be gradually withdrawn.
**Q: What withdrawal symptoms might occur when medication is stopped in cases of medication overuse headache?
Vomiting
Hypotension
Tachycardia
Restlessness
Sleep disturbances
Anxiety
**Q: What are common symptoms of meningitis and meningococcal disease?
Headache
Fever
Nausea/vomiting
Photophobia
Drowsiness
Seizures
**Q: What signs are commonly seen in meningitis and meningococcal disease?
Neck stiffness
Purpuric rash (particularly with invasive meningococcal disease)
**Q: What are the CSF findings in bacterial meningitis?
Appearance: Cloudy
Glucose: Low (< 1/2 plasma)
Protein: High (> 1 g/l)
White cells: 10 - 5,000 polymorphs/mm³
**Q: How does CSF in viral meningitis differ from bacterial meningitis?
Appearance: Clear/cloudy
Glucose: 60-80% of plasma glucose
Protein: Normal/raised
White cells: 15 - 1,000 lymphocytes/mm³
**Q: What are the CSF findings in tuberculous meningitis?
Appearance: Slight cloudy, fibrin web
Glucose: Low (< 1/2 plasma)
Protein: High (> 1 g/l)
White cells: 10 - 1,000 lymphocytes/mm³
**Q: What diagnostic methods are used in tuberculous meningitis when Ziehl-Neelsen stain is insufficient?
A: PCR (Polymerase Chain Reaction) is used, with a sensitivity of 75%, since Ziehl-Neelsen stain has only 20% sensitivity.
**Q: What atypical feature is associated with mumps and herpes encephalitis in terms of CSF findings?
A: A low glucose level may be seen in a proportion of cases of mumps and herpes encephalitis.
**Q: What are common neurological sequelae of meningitis?
Sensorineural hearing loss (most common)
Seizures
Focal neurological deficit
**Q: What are the infective complications of meningitis?
Sepsis
Intracerebral abscess
**Q: What are the pressure-related complications of meningitis?
Brain herniation
Hydrocephalus
**Q: What is Waterhouse-Friderichsen syndrome and its association with meningococcal meningitis?
A: Waterhouse-Friderichsen syndrome is adrenal insufficiency due to adrenal hemorrhage, and it is a risk in patients with meningococcal meningitis.
**Q: What are the key characteristics of a typical migraine?
Severe, unilateral, throbbing headache
Associated with nausea, photophobia, and phonophobia
Lasts up to 72 hours
Patients often go to a dark, quiet room during an attack
‘Classic’ migraines may be preceded by an aura
**Q: What is a typical aura in migraine patients?
Visual disturbance
Progressive
Lasts 5-60 minutes
Often characterized by transient hemianopic disturbance or a scintillating scotoma
**Q: What are common triggers for a migraine attack?
Tiredness, stress
Alcohol
Combined oral contraceptive pill
Lack of food or dehydration
Certain foods (cheese, chocolate, red wine, citrus fruits)
Menstruation
Bright lights
**Q: What are the diagnostic criteria for migraine according to the International Headache Society?
A. At least 5 attacks
B. Headaches lasting 4-72 hours
C. At least two of the following characteristics:
Unilateral location
Pulsating quality
Moderate/severe pain intensity
Aggravated by or causing avoidance of physical activity
D. At least one of the following during headache:
Nausea/vomiting
Photophobia/phonophobia
E. Not attributed to another disorder
**Q: What is hemiplegic migraine?
A variant of migraine with motor weakness as part of the aura
Affects around 0.01% of migraine patients
More common in adolescent females
Half of patients have a strong family history
Q: What are some key differences in migraine attacks for children according to diagnostic criteria?
Attacks may be shorter-lasting
Headache is more commonly bilateral
Gastrointestinal disturbance is more prominent
Q: How is migraine with aura defined and what percentage of patients experience it?
Migraine with aura occurs in around 25% of migraine patients
Aura is progressive in nature and may occur hours prior to the headache
Typical aura includes transient hemianopic disturbance or a spreading scintillating scotoma (‘jagged crescent’)
Sensory symptoms may also occur
Q: What are the differences between the International Headache Society and NICE criteria for migraine with aura?
NICE criteria:
Auras may occur with or without headache
Auras are fully reversible
Auras develop over at least 5 minutes and last 5-60 minutes
NICE also mentions:
Auras can be unilateral or bilateral
Atypical symptoms that may prompt further investigation: motor weakness, double vision, visual symptoms affecting only one eye, poor balance, decreased level of consciousness
Q: What are some atypical aura symptoms that may require further investigation or referral?
Motor weakness
Double vision
Visual symptoms affecting only one eye
Poor balance
Decreased level of consciousness
Q: What is the first-line acute treatment for migraines according to NICE guidelines?
Combination therapy:
An oral triptan and an NSAID, or
An oral triptan and paracetamol
Q: What is recommended for young people (aged 12-17 years) with migraine?
Consider a nasal triptan in preference to an oral triptan
Q: What should be done if first-line acute treatment for migraine is ineffective or not tolerated?
Offer a non-oral preparation of metoclopramide or prochlorperazine
Consider adding a non-oral NSAID or tripan
Q: What caution should be observed when prescribing metoclopramide for young patients?
Caution is needed as acute dystonic reactions may develop in young patients
Q: When should prophylactic treatment be considered for migraine?
If migraine attacks significantly impact quality of life and daily function, for example, if they occur frequently (more than once a week on average) or are severe despite optimal acute treatment
Q: What are the first-line options for migraine prophylaxis?
Propranolol
Topiramate (avoided in women of childbearing age due to teratogenicity and reduced effectiveness of hormonal contraceptives)
Amitriptyline
Q: What alternative treatment for migraine prophylaxis is recommended by NICE if other measures fail?
A course of up to 10 sessions of acupuncture over 5-8 weeks
Q: What is NICE’s recommendation for riboflavin in migraine management?
Riboflavin (400 mg once a day) may be effective in reducing migraine frequency and intensity for some people
Q: What is recommended for women with predictable menstrual migraine?
Frovatriptan (2.5 mg twice a day) or Zolmitriptan (2.5 mg twice or three times a day) as a type of ‘mini-prophylaxis’
Q: What is the first-line treatment for migraine during pregnancy?
Paracetamol (1g) is the first-line treatment
Q: What second-line treatments for migraine can be used during the first and second trimesters of pregnancy?
NSAIDs (non-steroidal anti-inflammatory drugs) can be used as a second-line treatment
Q: What medications should be avoided during pregnancy for migraine treatment?
Aspirin
Opioids such as codeine
Q: What is the contraindication for using the combined oral contraceptive (COC) pill in patients with migraine?
The COC pill is absolutely contraindicated in patients with migraine with aura due to an increased risk of stroke (relative risk 8.72)
Q: What treatment options are recommended for migraine during menstruation?
Mefanamic acid
A combination of aspirin, paracetamol, and caffeine
Triptans are recommended for acute situations
Q: What is the safety of prescribing hormone replacement therapy (HRT) for patients with a history of migraine?
It is safe to prescribe HRT for patients with a history of migraine, but it may make migraines worse.
Q: What is the most common presentation of Amyotrophic Lateral Sclerosis (ALS) in motor neuron disease?
Asymmetric limb weakness is the most common presentation of ALS.
Q: What signs are typically seen in motor neuron disease?
Mixture of upper and lower motor neuron signs
Wasting of small hand muscles or tibialis anterior
Fasciculations
Absence of sensory signs/symptoms (vague sensory symptoms like limb pain may occur early but never full sensory signs)
Q: What muscles are usually not affected in motor neuron disease?
External ocular muscles are not affected.
Q: What other features are typically not present in motor neuron disease?
No cerebellar signs
Abdominal reflexes are usually preserved
Sphincter dysfunction is a late feature
Q: How is the diagnosis of motor neuron disease made?
The diagnosis is clinical.
Nerve conduction studies show normal motor conduction, helping exclude a neuropathy.
Electromyography shows a reduced number of action potentials with increased amplitude.
MRI is used to exclude other diagnoses such as cervical cord compression and myelopathy.
Q: What is multiple sclerosis (MS)?
A: A chronic cell-mediated autoimmune disorder characterized by demyelination in the central nervous system.
Q: How much more common is MS in women compared to men?
A: 3 times more common in women.
Q: At what age is MS most commonly diagnosed?
A: Between 20-40 years.
Q: What is the most common form of MS?
A: Relapsing-remitting disease.
Q: What characterizes relapsing-remitting MS?
A: Acute attacks lasting 1-2 months followed by periods of remission.
Q: What percentage of MS patients have relapsing-remitting disease?
A: Around 85%.
Q: What is secondary progressive MS?
A: Describes relapsing-remitting patients who have deteriorated and developed neurological signs and symptoms between relapses.
Q: What symptoms are generally seen in secondary progressive MS?
A: Gait and bladder disorders.
Q: How does primary progressive MS present?
A: Progressive deterioration from onset.
Q: In which age group is primary progressive MS more common?
A: More common in older people.
Q: What is required for the diagnosis of multiple sclerosis?
Diagnosis requires demonstration of lesions disseminated in time and space.
Q: What does MRI show in multiple sclerosis?
High signal T2 lesions
Periventricular plaques
Dawson fingers: hyperintense lesions often seen on FLAIR images, perpendicular to the corpus callosum.
Q: What are the findings in CSF for multiple sclerosis?
Oligoclonal bands (present in CSF, not in serum).
Increased intrathecal synthesis of IgG.
Q: What do visual evoked potentials show in multiple sclerosis?
Delayed, but well-preserved waveform.
Q: What is the focus of treatment in multiple sclerosis?
Treatment is focused on reducing the frequency and duration of relapses.
There is no cure for multiple sclerosis.
Q: What is the management for an acute relapse in multiple sclerosis?
High-dose steroids (e.g., oral or IV methylprednisolone) for 5 days to shorten the length of the relapse.
Steroids shorten the duration of relapse but do not affect recovery (whether the patient returns to baseline function).
Q: When are disease-modifying drugs indicated in multiple sclerosis?
Relapsing-remitting disease with 2 relapses in past 2 years and able to walk 100m unaided.
Secondary progressive disease with 2 relapses in past 2 years and able to walk 10m (aided or unaided).
Q: What are the disease-modifying drug options for MS?
Natalizumab: monoclonal antibody, inhibits leucocyte migration across the blood-brain barrier, given intravenously.
Ocrelizumab: anti-CD20 monoclonal antibody, high-efficacy, given intravenously.
Fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes, available orally.
Beta-interferon: less effective, given subcutaneously/intramuscularly.
Glatiramer acetate: immunomodulating drug, acts as an immune decoy, given subcutaneously.
Q: How is fatigue managed in multiple sclerosis?
Trial of amantadine after excluding other causes (e.g., anaemia, thyroid problems, depression).
Mindfulness training and CBT may also be helpful.
Q: How is spasticity managed in multiple sclerosis?
First-line treatments: baclofen and gabapentin.
Other options: diazepam, dantrolene, tizanidine.
Physiotherapy is important.
Cannabis and botox are undergoing evaluation.
Q: How is bladder dysfunction managed in multiple sclerosis?
Ultrasound to assess bladder emptying first.
Intermittent self-catheterisation if significant residual volume.
Anticholinergics may help if no significant residual volume and can improve urinary frequency.
Q: How is oscillopsia (visual field oscillation) managed in multiple sclerosis?
Gabapentin is first-line for managing oscillopsia.
Q: What are the two predominant types of Multiple System Atrophy (MSA)?
MSA-P: Predominant Parkinsonian features.
MSA-C: Predominant Cerebellar features
Q: What is another name for a type of MSA?
Shy-Drager syndrome is a type of Multiple System Atrophy (MSA).
Q: What are common features of Multiple System Atrophy (MSA)?
Parkinsonism (similar to Parkinson’s disease)
Autonomic disturbance
Erectile dysfunction (often an early feature)
Postural hypotension
Atonic bladder
Cerebellar signs (e.g., ataxia)
Q: What is the key feature of Myasthenia Gravis?
Muscle fatigability: Muscles become progressively weaker during periods of activity and improve after rest.
Q: What are common symptoms of Myasthenia Gravis?
Extraocular muscle weakness: Diplopia (double vision)
Proximal muscle weakness: Face, neck, and limb girdle muscles
Ptosis
Dysphagia (difficulty swallowing)
Q: What are the associations of Myasthenia Gravis?
Thymomas: Found in 15% of cases
Autoimmune disorders: Pernicious anemia, autoimmune thyroid disorders, rheumatoid arthritis, SLE
Thymic hyperplasia: Found in 50-70% of cases
Q: What investigations are used to diagnose Myasthenia Gravis?
Single fiber electromyography: High sensitivity (92-100%)
CT thorax: To exclude thymoma
Antibodies to acetylcholine receptors: Positive in 85-90% of cases
Tensilon test: IV edrophonium temporarily improves muscle weakness (not commonly used due to cardiac arrhythmia risk)
Q: What is the first-line treatment for Myasthenia Gravis?
Pyridostigmine (long-acting acetylcholinesterase inhibitor)
Q: What immunosuppressive treatments may be used for Myasthenia Gravis?
Prednisolone (initially)
Azathioprine, Cyclosporine, Mycophenolate mofetil (may be used later)
Q: What are the management options for a Myasthenic crisis?
Plasmapheresis
Intravenous immunoglobulins (IVIg)
Q: What is the most common exacerbating factor in Myasthenia Gravis?
Exertion: Physical activity leads to increased fatigability, which is the hallmark feature of Myasthenia Gravis. Symptoms become more marked during the day.
Q: Which drugs can exacerbate Myasthenia Gravis?
Penicillamine
Quinidine and Procainamide
Beta-blockers
Lithium
Phenytoin
Antibiotics:
Gentamicin
Macrolides
Quinolones
Tetracyclines
Q: What are the two main types of Myotonic Dystrophy?
DM1 (Dystrophia Myotonica Type 1)
DM2 (Dystrophia Myotonica Type 2)
Q: What is the genetic basis of Myotonic Dystrophy?
Autosomal dominant inheritance
Trinucleotide repeat disorders
Q: What are the key differences between DM1 and DM2?
Q: What are the general features of Myotonic Dystrophy?
Myotonic facies (long, ‘haggard’ appearance)
Frontal balding
Bilateral ptosis
Cataracts
Dysarthria
Q: What are other common features of Myotonic Dystrophy?
Myotonia (tonic spasm of muscles)
Distal muscle weakness (initially in arms and legs)
Mild mental impairment
Diabetes mellitus
Testicular atrophy
Cardiac involvement (heart block, cardiomyopathy)
Dysphagia
Q: What is the association of Narcolepsy with HLA?
Narcolepsy is associated with HLA-DR2
Q: What protein is deficient in Narcolepsy?
Narcolepsy is associated with low levels of orexin (hypocretin), a protein responsible for controlling appetite and sleep patterns.
Q: What sleep pattern is early onset in Narcolepsy?
Early onset of REM sleep
Q: What is the typical onset age of Narcolepsy?
Typical onset is in teenage years
Q: What are the key features of Narcolepsy?
Hypersomnolence (excessive daytime sleepiness)
Cataplexy (sudden loss of muscle tone, often triggered by emotion)
Sleep paralysis
Vivid hallucinations (on going to sleep or waking up)
Q: What is the investigation for Narcolepsy?
Multiple sleep latency EEG
Q: What is the management for Narcolepsy?
Daytime stimulants (e.g., modafinil)
Nighttime sodium oxybate
Q: What are the two types of neurofibromatosis?
NF1 (von Recklinghausen’s syndrome)
NF2
Q: How are neurofibromatosis types inherited?
Both NF1 and NF2 are inherited in an autosomal dominant fashion.
Q: What are the common features of NF1?
Cafe-au-lait spots (≥ 6, 15 mm in diameter)
Axillary/groin freckles
Peripheral neurofibromas
Iris hamartomas (Lisch nodules) in > 90%
Scoliosis
Pheochromocytomas
Q: What are the common features of NF2?
Bilateral vestibular schwannomas
Multiple intracranial schwannomas, meningiomas, and ependymomas
Q: What is neuroleptic malignant syndrome (NMS)?
NMS is a rare but potentially fatal condition that can occur in patients taking antipsychotic medications or dopaminergic drugs (e.g., levodopa for Parkinson’s). It can happen when these medications are suddenly stopped or doses are reduced.
Q: What is the pathophysiology of neuroleptic malignant syndrome?
The exact pathophysiology is unknown, but one theory is that dopamine blockade by antipsychotics triggers massive glutamate release, leading to neurotoxicity and muscle damage.
Q: When does NMS typically occur after starting an antipsychotic?
NMS typically develops within hours to days of starting an antipsychotic medication.
Q: What are the classic features of neuroleptic malignant syndrome?
Pyrexia (fever)
Muscle rigidity
Autonomic lability (e.g., hypertension, tachycardia, tachypnoea)
Agitated delirium with confusion
Q: What lab findings are typically seen in NMS?
Raised creatine kinase (CK) (often markedly elevated)
Leukocytosis (elevated white blood cell count)
Acute kidney injury (secondary to rhabdomyolysis) in severe cases
Q: What is the management of neuroleptic malignant syndrome?
Stop antipsychotic medications
Transfer to a medical ward or intensive care unit
IV fluids to prevent renal failure
Dantrolene (helps decrease excitation-contraction coupling in skeletal muscle)
Bromocriptine (dopamine agonist) can also be used
Q: How does NMS differ from serotonin syndrome?
Both conditions can cause elevated creatine kinase (CK), but this is more strongly associated with NMS. NMS also tends to present with more muscle rigidity and autonomic instability.
Q: What is neuropathic pain?
Neuropathic pain is pain that arises following damage or disruption to the nervous system. It often responds poorly to standard analgesia.
Q: Can you name some examples of neuropathic pain conditions?
Diabetic neuropathy
Post-herpetic neuralgia
Trigeminal neuralgia
Prolapsed intervertebral disc
Q: What did NICE update in their 2013 guidance for the management of neuropathic pain?
First-line treatments include:
Amitriptyline
Duloxetine
Gabapentin
Pregabalin
If the first-line treatment does not work, another of the listed drugs should be tried.
When cant you use duloxetine
GFR <30
Q: How should drugs for neuropathic pain typically be used?
Drugs for neuropathic pain are typically used as monotherapy. If a drug does not work, it should be switched to another rather than added on top of the existing treatment.
Q: What medication can be used as ‘rescue therapy’ for exacerbations of neuropathic pain?
Tramadol may be used as rescue therapy.
Q: What is a treatment option for localized neuropathic pain such as post-herpetic neuralgia?
Topical capsaicin may be used for localized neuropathic pain.
Q: What can be beneficial for patients with resistant neuropathic pain problems?
Pain management clinics may be useful for patients with resistant neuropathic pain problems.
Q: What is the first-line treatment for trigeminal neuralgia?
Carbamazepine is used first-line for trigeminal neuralgia.
Q: What is normal pressure hydrocephalus (NPH)?
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients, often due to reduced CSF absorption at the arachnoid villi, which can be secondary to head injury, subarachnoid hemorrhage, or meningitis.
Q: What is the classical triad of features in normal pressure hydrocephalus?
Urinary incontinence
Dementia and bradyphrenia
Gait abnormality (may be similar to Parkinson’s disease)
Q: What imaging findings are characteristic of normal pressure hydrocephalus?
Imaging shows hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement.
Q: What is the primary management for normal pressure hydrocephalus?
Ventriculoperitoneal shunting is the primary management.
Q: What are some potential complications of ventriculoperitoneal shunting in NPH patients?
Around 10% of patients who have shunts experience significant complications such as seizures, infection, and intracerebral hemorrhages.
Q: What is Parkinson’s disease?
Parkinson’s disease is a progressive neurodegenerative condition caused by the degeneration of dopaminergic neurons in the substantia nigra, resulting in a classical triad of features: bradykinesia, tremor, and rigidity.
Q: What is the classical triad of features in Parkinson’s disease?
Bradykinesia
Tremor
Rigidity
Q: What are the features of bradykinesia in Parkinson’s disease?
Poverty of movement, also referred to as hypokinesia
Short, shuffling steps with reduced arm swinging
Difficulty in initiating movement
Q: Describe the tremor associated with Parkinson’s disease.
Most marked at rest, 3-5 Hz
Worse when stressed or tired, improves with voluntary movement
Typically ‘pill-rolling’, i.e., in the thumb and index finger
Q: What are the types of rigidity seen in Parkinson’s disease?
Lead pipe rigidity
Cogwheel rigidity: due to superimposed tremor
Q: List other characteristic features of Parkinson’s disease.
Mask-like facies
Flexed posture
Micrographia
Drooling of saliva
Psychiatric features: depression (most common), dementia, psychosis, and sleep disturbances
Impaired olfaction
REM sleep behavior disorder
Fatigue
Autonomic dysfunction, including postural hypotension
Q: How does drug-induced parkinsonism differ from Parkinson’s disease?
Motor symptoms are generally rapid onset and bilateral
Rigidity and rest tremor are uncommon
Q: How is Parkinson’s disease typically diagnosed?
Diagnosis is usually clinical. However, if there is difficulty differentiating between essential tremor and Parkinson’s disease, NICE recommends considering 123I-FP-CIT single photon emission computed tomography (SPECT).
Q: Who should diagnose and initiate the management of Parkinson’s disease?
A specialist with expertise in movement disorders should diagnose and initiate the management of Parkinson’s disease.
Q: What is the first-line treatment for motor symptoms affecting quality of life in Parkinson’s disease?
Levodopa
Q: What is the first-line treatment for motor symptoms not affecting quality of life in Parkinson’s disease?
Dopamine agonist (non-ergot derived)
Levodopa
Monoamine oxidase B (MAO-B) inhibitor
Q: What are the common side effects of levodopa?
Dry mouth
Anorexia
Palpitations
Postural hypotension
Psychosis
Q: What is the ‘on-off’ phenomenon associated with levodopa?
Large variations in motor performance, with normal function during the ‘on’ period, and weakness and restricted mobility during the ‘off’ period.
Q: What should be done if a patient continues to have symptoms despite optimal levodopa treatment?
NICE recommends the addition of a dopamine agonist, MAO-B inhibitor, or catechol-O-methyl transferase (COMT) inhibitor as an adjunct.
Q: What are dopamine receptor agonists, and what are their side effects?
Examples include bromocriptine, ropinirole, cabergoline, and apomorphine. Side effects include impulse control disorders, excessive daytime somnolence, hallucinations in older patients, nasal congestion, and postural hypotension.
Q: What are antimuscarinics, and what is their role in Parkinson’s disease?
Antimuscarinics block cholinergic receptors and are now more commonly used to treat drug-induced parkinsonism rather than idiopathic Parkinson’s disease. They help with tremor and rigidity (e.g., procyclidine, benzotropine, trihexyphenidyl).
Q: What should be considered if a patient with Parkinson’s disease develops orthostatic hypotension?
A medication review looking at potential causes should be done. If symptoms persist, midodrine can be considered.
Q: How should excessive daytime sleepiness be managed in Parkinson’s disease patients?
Patients should not drive, medication should be adjusted to control symptoms, and modafinil can be considered if alternative strategies fail.
Q: Why is it important not to acutely stop levodopa in Parkinson’s disease patients?
Acutely stopping levodopa can lead to acute akinesia or neuroleptic malignant syndrome. If a patient with Parkinson’s disease cannot take levodopa orally, they can be given a dopamine agonist patch as rescue medication to prevent acute dystonia.
Q: What is Parkinsonism?
A syndrome characterized by tremor, bradykinesia, rigidity, and postural instability, typically associated with Parkinson’s disease but can also have other causes.
Q: Name two drugs that can induce Parkinsonism.
Antipsychotics
Metoclopramide
Q: Which antiemetic does not cross the blood-brain barrier and does not cause extra-pyramidal side effects?
Domperidone
Q: What is progressive supranuclear palsy (PSP) and how is it related to Parkinsonism?
PSP is a neurodegenerative disease involving the gradual deterioration of cells in the brain, leading to symptoms of Parkinsonism.
Q: Name a neurodegenerative disorder other than Parkinson’s disease that can cause Parkinsonism.
Multiple system atrophy (MSA)
Q: What is Wilson’s disease and how does it relate to Parkinsonism?
Wilson’s disease is a genetic disorder in which copper builds up in the body, leading to neurological symptoms, including Parkinsonism.
Q: How can post-encephalitis lead to Parkinsonism?
Post-encephalitis Parkinsonism can occur after an inflammation of the brain, often due to viral infection, leading to damage in regions controlling movement.
Q: What is paroxysmal hemicrania (PH)?
A type of severe, unilateral headache that typically occurs in the orbital, supraorbital, or temporal region.
Q: How long do attacks of paroxysmal hemicrania usually last?
Less than 30 minutes.
Q: How often can paroxysmal hemicrania attacks occur in a day?
Multiple times a day.
Q: Which group of headache disorders does paroxysmal hemicrania belong to?
Trigeminal autonomic cephalgias.
Q: Name another condition within the trigeminal autonomic cephalgias group that shares many features with paroxysmal hemicrania.
Cluster headache.
Q: What is the distinguishing feature of paroxysmal hemicrania in terms of treatment response?
It is completely responsive to indomethacin.
Q: Name some conditions that cause predominantly motor loss in peripheral neuropathy.
Guillain-Barre syndrome, porphyria, lead poisoning, hereditary sensorimotor neuropathies (Charcot-Marie-Tooth), chronic inflammatory demyelinating polyneuropathy (CIDP), diphtheria.
Q: Name some conditions that cause predominantly sensory loss in peripheral neuropathy.
Diabetes, uraemia, leprosy, alcoholism, vitamin B12 deficiency, amyloidosis.
Q: In alcoholic neuropathy, which symptoms typically present first, sensory or motor?
Sensory symptoms.
Q: What is subacute combined degeneration of the spinal cord?
A condition seen in vitamin B12 deficiency where both the dorsal columns and the lateral corticospinal tracts of the spinal cord are affected.
Q: What are the acute adverse effects of phenytoin?
Dizziness
Diplopia
Nystagmus
Slurred speech
Ataxia
Confusion
Seizures (later)
Q: What are the chronic adverse effects of phenytoin?
Gingival hyperplasia
Hirsutism
Coarsening of facial features
Drowsiness
Megaloblastic anaemia
Peripheral neuropathy
Osteomalacia
Lymphadenopathy
Dyskinesia
Q: What are the teratogenic effects of phenytoin?
Phenytoin is associated with cleft palate and congenital heart disease in the offspring.
Q: When should phenytoin levels be monitored?
Phenytoin levels should be checked if:
Adjusting the phenytoin dose
Suspecting toxicity
Detecting non-adherence to the prescribed medication
Q: What condition is commonly associated with chronic phenytoin use and leads to facial changes?
Coarsening of facial features.
Q: What is a common adverse effect of phenytoin related to oral health?
Gingival hyperplasia, secondary to increased expression of platelet-derived growth factor (PDGF).
Q: What is pituitary apoplexy?
Pituitary apoplexy is the sudden enlargement of a pituitary tumor, usually a non-functioning macroadenoma, secondary to hemorrhage or infarction.
Q: What are some precipitating factors for pituitary apoplexy?
Hypertension
Pregnancy
Trauma
Anticoagulation
Q: What are the common features of pituitary apoplexy?
Sudden onset headache (similar to subarachnoid hemorrhage)
Vomiting
Neck stiffness
Visual field defects (classically bitemporal superior quadrantic defect)
Extraocular nerve palsies
Features of pituitary insufficiency (e.g., hypotension, hyponatraemia secondary to hypoadrenalism)
Q: What is the diagnostic investigation for pituitary apoplexy?
MRI is diagnostic.
Q: How is pituitary apoplexy managed?
Urgent steroid replacement due to loss of ACTH
Careful fluid balance
Surgery
Q: When does a post-lumbar puncture headache typically develop?
It usually develops within 24-48 hours after the lumbar puncture but may occur up to one week later.
Q: What are the typical characteristics of a post-lumbar puncture headache?
Worsens with an upright position
Improves with recumbent position
May last several days
Q: How is post-lumbar puncture headache managed?
Supportive initially (analgesia, rest)
If pain continues for more than 72 hours, specific treatments include: blood patch, epidural saline, and intravenous caffeine.
Q: What are the key features of Progressive Supranuclear Palsy?
Postural instability and falls
Stiff, broad-based gait
Impairment of vertical gaze (worse downward gaze)
Parkinsonism with prominent bradykinesia
Cognitive impairment, primarily frontal lobe dysfunction
Q: What specific problem do patients with Progressive Supranuclear Palsy face with their gaze?
Impairment of vertical gaze, with difficulty looking down more than up (difficulty reading or descending stairs).
Q: What is another name for Psychogenic Non-Epileptic Seizures?
Pseudoseizures.
Q: What are common factors that favor the diagnosis of pseudoseizures?
Pelvic thrusting
Family member with epilepsy
Much more common in females
Crying after seizure
Seizures don’t occur when alone
Gradual onset
Q: What factors favor the diagnosis of true epileptic seizures?
Tongue biting
Raised serum prolactin
Q: Why is serum prolactin raised following epileptic seizures?
It is hypothesized that the spread of electrical activity to the ventromedial hypothalamus triggers the release of a specific prolactin regulator into the hypophyseal portal system, although the exact mechanism is not fully understood.
Q: What is the root value of the radial nerve?
C5 to T1.
Q: What is the sensory function of the radial nerve?
It supplies the skin of the proximal phalanges on the dorsal aspect of the hand (excluding the little finger and part of the ring finger).
Q: What is the effect of radial nerve paralysis at different anatomical locations?
Shoulder (long head of triceps): Minor effect on shoulder stability in abduction
Arm (triceps): Loss of elbow extension
Forearm: Weakening of supination of the prone hand and elbow flexion in a mid-prone position
Q: What is the characteristic sign of radial nerve damage at the wrist?
Wrist drop and sensory loss to a small area between the dorsal aspect of the 1st and 2nd metacarpals.
Q: What happens in case of axillary damage to the radial nerve?
Paralysis of the triceps
Loss of elbow extension
Q: What is the normal range for intracranial pressure (ICP) in adults?
7-15 mmHg in adults in the supine position.
Q: What is the formula for cerebral perfusion pressure (CPP)?
CPP = Mean arterial pressure (MAP) - ICP
Q: What are the common causes of raised intracranial pressure (ICP)?
Idiopathic intracranial hypertension
Traumatic head injuries
Infection (e.g. meningitis)
Tumours
Hydrocephalus
Q: What are the key clinical features of raised ICP?
Headache
Vomiting
Reduced levels of consciousness
Papilloedema
Cushing’s triad (widening pulse pressure, bradycardia, irregular breathing)
Q: What is Cushing’s triad?
Widening pulse pressure
Bradycardia
Irregular breathing
Q: What are the primary investigations and monitoring techniques for raised ICP?
Neuroimaging (CT/MRI)
Invasive ICP monitoring (via catheter placed in the lateral ventricles
Q: What ICP value is typically used to determine the need for treatment?
20 mmHg
Q: What is the initial management approach for raised ICP?
Investigate and treat the underlying cause
Head elevation to 30º
IV mannitol (osmotic diuretic)
Controlled hyperventilation (to reduce pCO2 and vasoconstrict cerebral arteries)
Removal of CSF (via intraventricular drainage, repeated lumbar puncture, or ventriculoperitoneal shunt for hydrocephalus)
Q: What spinal root corresponds to the ankle reflex?
S1-S2
Q: What spinal root corresponds to the knee reflex?
L3-L4
Q: What spinal root corresponds to the biceps reflex?
C5-C6
Q: What spinal root corresponds to the triceps reflex?
C7-C8
Q: What is the primary symptom of Restless Legs Syndrome (RLS)?
Uncontrollable urge to move the legs (akathisia), often accompanied by paraesthesias like crawling or throbbing sensations.
Q: When do symptoms of RLS typically occur?
Symptoms initially occur at night but may also occur during the day as the condition progresses.
Q: What is a common observation related to sleep in patients with RLS?
Periodic limb movements of sleep (PLMS), which may be noted by a partner.
Q: How is RLS diagnosed?
The diagnosis is clinical, although blood tests such as ferritin to exclude iron deficiency anaemia may be useful.
Q: What are the first-line treatments for RLS?
Dopamine agonists (e.g., pramipexole, ropinirole)
Benzodiazepines
Gabapentin
Q: What simple measures can help manage RLS?
Walking
Stretching
Massaging the affected limbs
Q: What is Reye’s syndrome?
A severe, progressive encephalopathy affecting children, accompanied by fatty infiltration of the liver, kidneys, and pancreas.
Q: What is a notable association with Reye’s syndrome?
There is a known association with aspirin use, and a viral cause has been postulated.
Q: What are the key features of Reye’s syndrome?
A history of preceding viral illness
Encephalopathy: confusion, seizures, cerebral oedema, coma
Fatty infiltration of the liver, kidneys, and pancreas
Hypoglycaemia
Q: What is the management of Reye’s syndrome?
Management is supportive.
Q: What is the initial approach to managing a patient having a seizure?
Check the airway and apply oxygen if appropriate.
Place the patient in the recovery position.
If the seizure is prolonged, administer benzodiazepines.
Q: What is the recommended dose of rectal diazepam for neonates and children according to the BNF?
Neonate: 1.25 - 2.5 mg
Child 1 month - 1 year: 5 mg
Child 2 - 11 years: 5 - 10 mg
Child 12 - 17 years: 10 mg
Adult: 10 - 20 mg (max. 30 mg)
Elderly: 10 mg (max. 15 mg)
Q: What is the recommended dose of midazolam oromucosal solution for neonates and children?
Neonate: 300 mcg/kg (unlicensed)
Child 1 - 2 months: 300 mcg/kg (max. 2.5 mg, unlicensed)
Child 3 - 11 months: 2.5 mg
Child 1 - 4 years: 5 mg
Child 5 - 9 years: 7.5 mg
Child 10 - 17 years: 10 mg
Adult: 10 mg (unlicensed)
Q: When is repeated administration of diazepam recommended in seizure management?
Diazepam may be repeated once after 10-15 minutes if necessary.
Q: What is the primary use of sodium valproate?
Sodium valproate is primarily used in the management of epilepsy and is first-line therapy for generalized seizures. It works by increasing GABA activity.
Q: What are the teratogenic effects of sodium valproate?
Sodium valproate is teratogenic and is associated with neural tube defects. Maternal use increases the risk of neurodevelopmental delay in children. It should not be used during pregnancy or in women of childbearing age unless absolutely necessary and only under specialist advice.
Q: What are the gastrointestinal side effects of sodium valproate?
Common gastrointestinal side effects include nausea and increased appetite, which may lead to weight gain.
Q: What are some other side effects of sodium valproate?
Alopecia (regrowth may be curly)
Ataxia
Tremor
Hepatotoxicity
Pancreatitis
Thrombocytopaenia
Hyponatraemia
Hyperammonemic encephalopathy (can be treated with L-carnitine)
Q: What is spastic paraparesis?
Spastic paraparesis refers to upper motor neuron weakness in the lower limbs, characterized by spasticity and weakness.
Q: What are some causes of spastic paraparesis?
Demyelination: e.g., multiple sclerosis
Cord compression: trauma, tumour
Parasagittal meningioma
Tropical spastic paraparesis
Transverse myelitis: e.g., HIV
Syringomyelia
Hereditary spastic paraplegia
Osteoarthritis of the cervical spine
Q: What is the motor lesion caused by amyotrophic lateral sclerosis (ALS)?
ALS affects both upper and lower motor neurons, leading to a combination of upper motor neuron signs (e.g., spasticity) and lower motor neuron signs (e.g., muscle weakness, atrophy).
Q: What is the motor lesion caused by poliomyelitis?
Poliomyelitis affects the anterior horns of the spinal cord, resulting in lower motor neuron signs such as muscle weakness and atrophy.
Q: What are the key features of Brown-Sequard syndrome?
Lateral corticospinal tract: Ipsilateral spastic paresis below the lesion
Dorsal columns: Ipsilateral loss of proprioception and vibration sensation
Lateral spinothalamic tract: Contralateral loss of pain and temperature sensation
Q: What is subacute combined degeneration of the spinal cord and its features?
This condition is caused by vitamin B12 and E deficiencies, affecting:
Lateral corticospinal tracts: Bilateral spastic paresis
Dorsal columns: Bilateral loss of proprioception and vibration sensation
Spinocerebellar tracts: Bilateral limb ataxia
Q: What is the main difference between subacute combined degeneration and Friedrich’s ataxia?
Both conditions affect the same spinal tracts, but Friedrich’s ataxia also causes cerebellar ataxia and other symptoms such as intention tremor.
Q: What is the effect of anterior spinal artery occlusion?
Lateral corticospinal tracts: Bilateral spastic paresis
Lateral spinothalamic tracts: Bilateral loss of pain and temperature sensation
Q: What is syringomyelia and its clinical features?
Syringomyelia affects the ventral horns and lateral spinothalamic tract, leading to:
Flaccid paresis (typically affecting the intrinsic hand muscles)
Loss of pain and temperature sensation
Q: What is the clinical presentation of multiple sclerosis in terms of spinal cord lesions?
Multiple sclerosis involves asymmetrical damage to varying spinal tracts, leading to a combination of motor, sensory, and ataxia symptoms.
Q: What is the sensory lesion caused by neurosyphilis (tabes dorsalis)?
Neurosyphilis affects the dorsal columns, leading to loss of proprioception and vibration sensation.
Q: What is the cause of spontaneous intracranial hypotension?
Spontaneous intracranial hypotension results from a cerebrospinal fluid (CSF) leak, often from the thoracic nerve root sleeves.
Q: What are the risk factors for spontaneous intracranial hypotension?
Risk factors include connective tissue disorders such as Marfan’s syndrome.
Q: What is the typical feature of the headache in spontaneous intracranial hypotension?
The headache has a strong postural relationship, being much worse when upright. Patients may be bed-bound due to this headache.
Q: What is the key imaging finding in spontaneous intracranial hypotension?
MRI with gadolinium typically shows pachymeningeal enhancement.
Q: How is spontaneous intracranial hypotension generally managed?
Management is usually conservative, but if this fails, an epidural blood patch may be used.
Q: How is status epilepticus defined?
A single seizure lasting >5 minutes, or
≥ 2 seizures within a 5-minute period without the person returning to normal between them.
Q: What is the first-line treatment for status epilepticus?
First-line drugs are benzodiazepines. In the prehospital setting, PR diazepam or buccal midazolam may be given. In hospital, IV lorazepam is generally used, with the option to repeat it after 5-10 minutes.
Q: What second-line drugs are used in status epilepticus if seizures persist?
If seizures continue, second-line agents such as levetiracetam, phenytoin, or sodium valproate are used.
Q: What should be done if status epilepticus is refractory (no response) after 45 minutes?
If no response after 45 minutes, induction of general anaesthesia or phenobarbital should be used to achieve rapid control of seizure activity.
Q: What are the effects of a lesion in the anterior cerebral artery?
Contralateral hemiparesis and sensory loss, with lower extremity > upper.
Q: What are the effects of a lesion in the middle cerebral artery?
Contralateral hemiparesis and sensory loss, with upper extremity > lower.
Contralateral homonymous hemianopia.
Aphasia
Q: What are the effects of a lesion in the posterior cerebral artery?
Contralateral homonymous hemianopia with macular sparing.
Visual agnosia.
Q: What is the presentation of Weber’s syndrome (caused by branches of the posterior cerebral artery)?
Ipsilateral CN III palsy.
Contralateral weakness of the upper and lower extremity.
Q: What are the effects of a lesion in the posterior inferior cerebellar artery (lateral medullary syndrome/Wallenberg syndrome)?
Ipsilateral: facial pain and temperature loss.
Contralateral: limb/torso pain and temperature loss.
Ataxia, nystagmus.
Q: What are the effects of a lesion in the anterior inferior cerebellar artery (lateral pontine syndrome)?
Symptoms similar to Wallenberg’s (lateral medullary syndrome) but with:
Ipsilateral facial paralysis and deafness.
Q: What is amaurosis fugax and which artery is associated with it?
Amaurosis fugax is temporary vision loss, associated with a retinal/ophthalmic artery lesion.
Q: What is the presentation of basilar artery stroke?
‘Locked-in syndrome’ (complete paralysis except for eye movements).
Q: What are the common presentations of lacunar strokes?
Isolated hemiparesis, hemisensory loss, or hemiparesis with limb ataxia.
Strong association with hypertension.
Common sites: basal ganglia, thalamus, and internal capsule.
Q: What is the recommended fluid management for acute stroke patients?
Regularly assess hydration to maintain normovolaemia.
Oral hydration is preferable for patients able to swallow safely.
If necessary, use isotonic saline without dextrose for intravenous hydration.
Be cautious of both hypovolaemia and over-hydration.
Q: What are the blood glucose management recommendations for acute stroke patients?
Aim to maintain blood glucose levels between 4-11 mmol/L.
Intensive management is needed for diabetic patients, using intravenous insulin and glucose infusions.
Avoid hypoglycaemia as it can cause neuronal injury and mimic stroke deficits.
Q: What is the Barthel index (BI) used for in stroke management?
The Barthel index measures functional status and disability after stroke.
It assesses 10 tasks such as feeding, bathing, dressing, and mobility.
Scores range from 0 (completely dependent) to 100 (completely independent), and it is used to monitor recovery and rehabilitation progress.
Q: What are the two main types of strokes?
Ischaemic Stroke – caused by a blockage in the blood vessel.
Subtypes: Thrombotic (from large vessels like the carotid) and Embolic (caused by blood clots or other materials).
TIA (Transient Ischaemic Attack) is a subtype with temporary symptoms.
Haemorrhagic Stroke – caused by a blood vessel bursting, leading to bleeding in the brain.
Subtypes: Intracerebral haemorrhage and Subarachnoid haemorrhage.
Q: What are the key symptoms of a stroke?
Motor weakness
Speech problems (dysphasia)
Swallowing problems
Visual field defects (e.g., homonymous hemianopia)
Balance problems
Brainstem infarctions may cause more severe symptoms like quadriplegia or lock-in syndrome.
Q: What are the symptoms more likely in haemorrhagic strokes?
Decreased level of consciousness (seen in up to 50% of patients)
Headache
Nausea and vomiting
Seizures (in up to 25% of patients)
Q: What imaging methods are used in acute stroke diagnosis?
CT scan
MRI
These help differentiate between ischaemic and haemorrhagic strokes, aiding treatment decisions like thrombolysis.
Q: What is the management for Ischaemic Strokes?
Thrombolysis if criteria are met (e.g., within 4.5 hours of symptom onset).
Aspirin 300mg as soon as possible after ruling out haemorrhagic stroke.
Continue antiplatelet therapy.
Q: How is a Transient Ischaemic Attack (TIA) managed?
Immediate aspirin 300mg unless contraindicated.
If recurrent TIAs or suspected cardioembolism, consult a stroke specialist urgently.
If suspected TIA occurred within the last 7 days, arrange urgent assessment within 24 hours.
Q: How is Haemorrhagic Stroke managed?
Consult neurosurgical team for possible interventions.
Stop anticoagulants and antithrombotics to minimize bleeding.
Reverse anticoagulation if necessary and manage blood pressure acutely to improve outcomes.
Q: What is the ROSIER score used for in stroke assessment?
The ROSIER score helps healthcare professionals assess the likelihood of stroke.
A score > 0 suggests that a stroke is likely.
Q: What is the first-line investigation for suspected stroke?
A non-contrast CT head scan is the first-line radiological investigation for suspected stroke. It helps differentiate between ischaemic and haemorrhagic strokes and detect other possible pathologies like a tumour.
Q: What signs on a CT scan indicate an acute ischaemic stroke?
Low density areas in the grey and white matter of the affected brain territory.
The ‘hyperdense artery’ sign, which represents a clot in the responsible artery, often visible immediately.
Q: What does a CT scan show in an acute haemorrhagic stroke?
Hyperdense material (blood) within the brain.
Surrounding low-density areas due to oedema.
This is a characteristic feature of haemorrhagic strokes.
Q: How should blood pressure be managed in the acute phase of ischaemic stroke?
Blood pressure should not be lowered unless there are complications (e.g., hypertensive encephalopathy) or if the patient is being considered for thrombolysis.
Blood pressure control should be considered if systolic BP > 150 mmHg within 6 hours of symptom onset.
Q: What medication should be administered if a haemorrhagic stroke has been excluded?
Aspirin 300 mg orally or rectally should be given as soon as possible.
Q: When can anticoagulants be started in patients with atrial fibrillation after an ischaemic stroke?
Anticoagulants should not be started until brain imaging has excluded haemorrhage and typically not until 14 days after an ischaemic stroke.
Q: What should be done if cholesterol is > 3.5 mmol/l in an acute ischaemic stroke patient?
The patient should be started on a statin, although many physicians delay treatment for at least 48 hours due to the risk of haemorrhagic transformation.
Q: What are the general criteria for thrombolysis with alteplase or tenecteplase in acute ischaemic stroke?
Administered within 4.5 hours of stroke symptom onset.
Haemorrhage has been excluded via imaging.
Q: What is the broader guideline for thrombolysis in acute ischaemic stroke, according to the 2023 National Clinical Guideline?
Patients with an acute ischaemic stroke who were last known to be well more than 4.5 hours ago can be considered for thrombolysis if:
Treatment can be started between 4.5 and 9 hours of onset or within 9 hours of waking up with symptoms, and
Imaging shows potential to salvage brain tissue (core-perfusion mismatch or DWI-FLAIR mismatch).
Q: What is the target blood pressure before administering thrombolysis?
Blood pressure should be lowered to 185/110 mmHg before thrombolysis.
Q: What are some absolute contraindications to thrombolysis in acute ischaemic stroke?
Previous intracranial haemorrhage
Seizure at onset of stroke
Intracranial neoplasm
Active bleeding
Uncontrolled hypertension (>200/120 mmHg)
Gastrointestinal haemorrhage in the past 3 weeks
Recent stroke/brain injury in the past 3 months
Q: What is the role of mechanical thrombectomy in acute ischaemic stroke?
Mechanical thrombectomy should be offered to patients with confirmed proximal anterior circulation occlusion (via CTA or MRA) within 6 hours of symptom onset, and within 24 hours for those with potential salvageable brain tissue, as shown by imaging.
Thrombectomy can be combined with intravenous thrombolysis if within 4.5 hours.
Q: What is the recommended secondary prevention after an ischaemic stroke?
Clopidogrel is preferred over combination aspirin and modified-release dipyridamole for secondary prevention.
Aspirin should be used only if clopidogrel is contraindicated or not tolerated.
Q: When should carotid endarterectomy be performed after an ischaemic stroke or TIA?
Recommended for patients with >50% stenosis in the carotid artery, especially in those who are not severely disabled.
It should be performed as soon as possible within 7 days of the stroke or TIA.
Q: What is a Total Anterior Circulation Infarct (TACI) and its characteristics?
Involves middle and anterior cerebral arteries.
All three criteria (hemiparesis, homonymous hemianopia, and higher cognitive dysfunction) are present.
Occurs in approximately 15% of strokes.
Q: What is a Partial Anterior Circulation Infarct (PACI) and its characteristics?
Involves smaller arteries of the anterior circulation (e.g., upper or lower division of the middle cerebral artery).
Two of the three criteria are present.
Occurs in approximately 25% of strokes.
Q: What is a Lacunar Infarct (LACI) and its characteristics?
Involves perforating arteries around the internal capsule, thalamus, and basal ganglia.
Presents with one of the following:
Unilateral weakness (and/or sensory deficit) of face and arm, arm and leg, or all three.
Pure sensory stroke.
Ataxic hemiparesis.
Occurs in approximately 25% of strokes.
Q: What is a Posterior Circulation Infarct (POCI) and its characteristics?
Involves the vertebrobasilar arteries.
Presents with one of the following:
Cerebellar or brainstem syndromes.
Loss of consciousness.
Isolated homonymous hemianopia.
Occurs in approximately 25% of strokes.Q: What is Lateral Medullary Syndrome (Wallenberg’s Syndrome) and its clinical features?
Q: What is Lateral Medullary Syndrome (Wallenberg’s Syndrome) and its clinical features?
Involves the posterior inferior cerebellar artery.
Ipsilateral symptoms: ataxia, nystagmus, dysphagia, facial numbness, cranial nerve palsy (e.g., Horner’s syndrome).
Contralateral symptoms: limb sensory loss.
Q: What is Weber’s Syndrome and its clinical features?
Ipsilateral oculomotor nerve (III) palsy.
Contralateral weakness (hemiparesis).
Q: What is the cause of Subacute Combined Degeneration of the Spinal Cord (SCD) and what tracts are involved?
Cause: Vitamin B12 deficiency (can also be caused by recreational nitrous oxide inhalation).
Tracts involved:
Dorsal columns
Lateral corticospinal tracts
Spinocerebellar tracts
Q: What are the features of dorsal column involvement in Subacute Combined Degeneration of the Spinal Cord?
Distal tingling/burning/sensory loss: Symmetrical, more prominent in legs than arms.
Impaired proprioception and vibration sense.
Q: What are the features of lateral corticospinal tract involvement in Subacute Combined Degeneration of the Spinal Cord?
Muscle weakness
Hyperreflexia and spasticity
Upper motor neuron signs: Typically develop in the legs first.
Brisk knee reflexes, absent ankle jerks, extensor plantars.
Q: What are the features of spinocerebellar tract involvement in Subacute Combined Degeneration of the Spinal Cord?
Sensory ataxia: Gait abnormalities.
Positive Romberg’s sign.
Q: What is Syringomyelia?
Syringomyelia is a collection of cerebrospinal fluid (CSF) within the spinal cord.
Syringobulbia is a similar condition with a fluid-filled cavity in the medulla of the brainstem.
Q: What are the causes of Syringomyelia?
Chiari malformation: Strong association
Trauma
Tumours
Idiopathic
Q: What are the features of Syringomyelia?
‘Cape-like’ distribution of sensory loss (neck, shoulders, and arms)
Loss of temperature sensation, but preservation of light touch, proprioception, and vibration
Spastic weakness (mainly in lower limbs)
Neuropathic pain
Upgoing plantars
Autonomic features:
Rare Horner’s syndrome (due to sympathetic chain compression)
Bowel and bladder dysfunction
Scoliosis (if untreated)
Q: What investigations are used for diagnosing Syringomyelia?
Full spine MRI with contrast to exclude a tumor or tethered cord.
Brain MRI to exclude a Chiari malformation
Q: What is the treatment for Syringomyelia?
Treatment focuses on addressing the underlying cause of the syrinx.
If the syrinx is persistent or symptomatic, a shunt may be placed to drain the fluid.
Q: What is Tension-type headache?
Tension-type headache is an episodic primary headache characterized by a tight band or pressure sensation around the head.
Symptoms are typically bilateral, unlike migraines, which are often unilateral.
It is usually of lower intensity than a migraine and is not associated with aura, nausea, vomiting, or aggravated by routine physical activity.
Q: What are the characteristic features of Tension-type headache?
Described as a tight band or pressure sensation around the head.
Bilateral headache, unlike unilateral migraines.
Lower intensity than migraines.
No aura, no nausea/vomiting, and not aggravated by physical activity.
May be associated with stress.
Can co-exist with migraine.
Q: What is Chronic tension-type headache?
Defined as tension-type headaches occurring on 15 or more days per month.
Q: What are the acute treatments for Tension-type headache according to NICE guidelines (2012)?
Aspirin, paracetamol, or NSAIDs are first-line treatments for acute episodes of tension-type headache.
Q: What are the prophylactic treatments for Tension-type headache according to NICE guidelines (2012)?
Acupuncture: Up to 10 sessions over 5-8 weeks.
Low-dose amitriptyline: Though widely used in the UK, NICE did not support pharmacological prophylaxis for tension-type headaches due to insufficient evidence. They suggest that the assessment may reveal coexisting migraine, leading to a possible diagnosis of chronic migraine.
Q: What are the features of Third Nerve Palsy?
Eye deviated “down and out”.
Ptosis (drooping eyelid).
Pupil may be dilated (sometimes referred to as a “surgical” third nerve palsy).
Q: What is Thoracic Outlet Syndrome (TOS) and what structures are commonly compressed?
TOS is a disorder involving compression of the brachial plexus, subclavian artery, or subclavian vein at the site of the thoracic outlet.
Neurogenic TOS accounts for 90% of cases.
Q: What is a Transient Ischaemic Attack (TIA) and how is it defined?
A TIA is a brief period of neurological deficit due to a vascular cause, typically lasting less than an hour.
The new definition is tissue-based: a transient episode of neurologic dysfunction caused by focal brain, spinal cord, or retinal ischaemia, without acute infarction.
Q: What are the clinical features of Transient Ischaemic Attack (TIA)?
Sudden onset of focal neurological deficits that resolve typically within 1 hour.
Unilateral weakness or sensory loss.
Aphasia or dysarthria.
Ataxia, vertigo, or loss of balance.
Visual problems:
Amaurosis fugax (sudden transient loss of vision in one eye).
Diplopia or homonymous hemianopia.
Q: What should be done in the assessment and referral of a patient with suspected TIA?
Aspirin 300 mg should be given immediately unless contraindicated.
The patient should be assessed urgently within 24 hours by a stroke specialist clinician.
Q: What are some TIA mimics that need to be excluded?
Hypoglycaemia
Intracranial haemorrhage (patients on anticoagulants or with similar risk factors should be admitted for urgent imaging to exclude haemorrhage).
Q: How is TIA diagnosed and what imaging is preferred?
MRI (including diffusion-weighted and blood-sensitive sequences) is preferred to determine the territory of ischaemia or detect haemorrhage/alternative pathologies.
CT is not recommended unless there is clinical suspicion of an alternative diagnosis like haemorrhage.
Q: What is the management for TIA?
Immediate antithrombotic therapy (if no contraindication to bleeding):
Aspirin 300 mg for resolved TIA symptoms, clopidogrel + aspirin for 21 days for high-risk patients.
After 21 days, clopidogrel 75 mg daily for long-term secondary prevention.
For atrial fibrillation, anticoagulation should be started once intracranial haemorrhage is excluded.
Lipid modification with a high-intensity statin (e.g., atorvastatin 20-80 mg daily) to reduce non-HDL cholesterol by more than 40%.
Q: What further investigations should be considered in TIA patients?
Carotid imaging (carotid duplex ultrasound, CT angiography, or MR angiography) should be done if carotid atherosclerosis is suspected, especially if a carotid intervention is considered.
Carotid endarterectomy is recommended for patients with symptomatic carotid stenosis (>50% stenosis according to NASCET criteria).
Carotid imaging should be performed within 24 hours of assessment.
Q: What are the updated guidelines for dual antiplatelet therapy (DAPT) in TIA management?
DAPT (clopidogrel + aspirin) is recommended for patients with TIA or minor ischaemic stroke within 24 hours of onset, provided there is no high risk of bleeding.
Clopidogrel (300 mg loading dose followed by 75 mg daily) + Aspirin (300 mg loading dose followed by 75 mg daily) for 21 days, then monotherapy with clopidogrel.
Ticagrelor + clopidogrel can be an alternative.
Q: What are the phases for typical TIA management?
Resolved TIA symptoms, awaiting specialist review: Aspirin.
Reviewed by specialist, initial 21 days (high risk): Aspirin + Clopidogrel.
Long-term secondary prevention (after 21 days): Clopidogrel.
Q: What is Trigeminal Neuralgia?
A pain syndrome characterised by severe unilateral pain.
The pain is described as brief electric shock-like pains, abrupt in onset and termination.
Limited to one or more divisions of the trigeminal nerve.
Often triggered by light touch (e.g., washing, shaving, smoking, talking, brushing teeth).
Pain may occur spontaneously and remit for variable periods.
Q: What are trigger factors for Trigeminal Neuralgia pain?
Light touch, such as from:
Washing,
Shaving,
Smoking,
Talking,
Brushing teeth.
Q: What are red flag symptoms that suggest a serious underlying cause of Trigeminal Neuralgia?
Sensory changes,
Deafness or other ear problems,
History of skin or oral lesions that could spread perineurally,
Pain only in the ophthalmic division (eye socket, forehead, and nose), or bilateral pain,
Optic neuritis,
Family history of multiple sclerosis,
Onset of symptoms before 40 years of age.
Q: What is the first-line treatment for Trigeminal Neuralgia?
Carbamazepine is the first-line treatment.
Q: What should be done if Trigeminal Neuralgia does not respond to treatment or presents with atypical features?
Refer the patient to neurology if there is failure to respond to treatment or atypical features (e.g., onset < 50 years old).
Q: What is the primary use of Triptans?
Triptans are specific 5-HT1B and 5-HT1D agonists used in the acute treatment of migraine.
Typically used first-line in combination therapy with an NSAID or paracetamol.
Q: When should Triptans be taken in relation to a migraine?
Triptans should be taken as soon as possible after the onset of headache, rather than at the onset of aura.
Q: What are the common adverse effects of Triptans?
‘Triptan sensations’:
Tingling,
Heat,
Tightness (e.g., throat and chest),
Heaviness,
Pressure.
Q: What are the contraindications for Triptans?
Patients with a history of, or significant risk factors for ischaemic heart disease or cerebrovascular disease should avoid Triptans.
Q: What type of inheritance pattern does Tuberous sclerosis (TS) follow?
Tuberous sclerosis is an autosomal dominant genetic condition.
Q: What are some cutaneous features of Tuberous sclerosis?
Depigmented ‘ash-leaf’ spots that fluoresce under UV light.
Shagreen patches (roughened patches of skin over lumbar spine).
Adenoma sebaceum (angiofibromas) in a butterfly distribution over the nose.
Fibromata beneath the nails (subungual fibromata).
Cafe-au-lait spots (less common but may be seen, found in 28% of patients with TS).
Q: What are some neurological features of Tuberous sclerosis?
Developmental delay.
Epilepsy, including infantile spasms or partial seizures.
Intellectual impairment.
Q: What are some additional features seen in Tuberous sclerosis?
Retinal hamartomas: dense white areas on retina (phakomata).
Rhabdomyomas of the heart.
Gliomatous changes in brain lesions.
Polycystic kidneys and renal angiomyolipomata.
Lymphangioleiomyomatosis: multiple lung cysts.
Q: From which part of the brachial plexus does the ulnar nerve arise?
The ulnar nerve arises from the medial cord of the brachial plexus (C8, T1).
Q: What sensory functions are controlled by the ulnar nerve?
Medial 1 1/2 fingers (palmar and dorsal aspects).
Q: What are the patterns of damage to the ulnar nerve at the wrist?
Claw hand: Hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits.
Wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals).
Wasting and paralysis of hypothenar muscles.
Sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects).
Q: What are the patterns of damage to the ulnar nerve at the elbow?
Same as damage at wrist (claw hand, wasting, and sensory loss), but with an ulnar paradox where the clawing is more severe in distal lesions.
Radial deviation of the wrist
Q: What is the classical history of a vestibular schwannoma?
Vertigo,
Hearing loss,
Tinnitus,
Absent corneal reflex.
Q: What condition is associated with bilateral vestibular schwannomas?
Neurofibromatosis type 2.
Q: What is the investigation of choice for suspected vestibular schwannomas?
MRI of the cerebellopontine angle is the investigation of choice.
Audiometry is also important, as 5% of patients will have a normal audiogram.
Q: How are vestibular schwannomas typically managed?
Surgery,
Radiotherapy,
Observation (since they are often slow-growing and benign).
Q: What is left homonymous hemianopia?
Left homonymous hemianopia refers to a visual field defect where the left side of the visual field is lost in both eyes, caused by a lesion of the right optic tract.
Q: What are homonymous quadrantanopias and their associated lesions?
Homonymous quadrantanopias involve visual field loss in one quadrant of both eyes.
Superior quadrantanopia: Lesion of the inferior optic radiations in the temporal lobe (Meyer’s loop).
Inferior quadrantanopia: Lesion of the superior optic radiations in the parietal lobe.
Mnemonic: PITS (Parietal-Inferior, Temporal-Superior).
Q: What does incongruous defects in visual fields indicate?
Incongruous defects are asymmetric or incomplete visual field losses, typically due to a lesion of the optic tract.
Q: What does congruous defects in visual fields indicate?
Congruous defects refer to symmetrical or complete visual field loss, typically due to a lesion of the optic radiation or occipital cortex.
Q: What is macula sparing in visual field defects?
Macula sparing suggests a lesion of the occipital cortex, where the central visual field (macula) remains intact.
Q: What lesion causes bitemporal hemianopia?
Lesion of the optic chiasm causes bitemporal hemianopia, where both outer fields are lost in both eyes.
Q: What causes upper quadrant defect > lower quadrant defect in bitemporal hemianopia?
Inferior chiasmal compression, commonly caused by a pituitary tumour.
Q: What causes lower quadrant defect > upper quadrant defect in bitemporal hemianopia?
Superior chiasmal compression, commonly caused by a craniopharyngioma.
Q: What is the inheritance pattern of Von Hippel-Lindau (VHL) syndrome?
Von Hippel-Lindau syndrome is an autosomal dominant condition.
Q: What are the common neoplastic features of Von Hippel-Lindau syndrome?
Cerebellar haemangiomas (may cause subarachnoid haemorrhages).
Retinal haemangiomas (can lead to vitreous haemorrhage).
Renal cysts (premalignant).
Phaeochromocytoma.
Extra-renal cysts (epididymal, pancreatic, hepatic).
Endolymphatic sac tumours.
Clear-cell renal cell carcinoma.
Q: What can cerebellar haemangiomas in Von Hippel-Lindau syndrome lead to?
Cerebellar haemangiomas can cause subarachnoid haemorrhages.
Q: What ocular finding is characteristic of Von Hippel-Lindau syndrome?
Retinal haemangiomas, which can lead to vitreous haemorrhage.
Q: What renal findings are associated with Von Hippel-Lindau syndrome?
Renal cysts (often premalignant) are commonly found in patients with VHL.
Q: What types of renal cancer are associated with Von Hippel-Lindau syndrome?
Clear-cell renal cell carcinoma is a notable risk in patients with VHL.
Q: What is the primary cause of Wernicke’s encephalopathy?
Wernicke’s encephalopathy is caused by thiamine deficiency, most commonly seen in alcoholics.
Other rare causes include persistent vomiting, anorexia nervosa, stomach cancer, and dietary deficiency.
Q: What is the classic triad of symptoms in Wernicke’s encephalopathy?
Oculomotor dysfunction (nystagmus, ophthalmoplegia).
Ataxia (gait disturbances).
Encephalopathy (confusion, disorientation, inattentiveness).
Q: What are the key ocular signs in Wernicke’s encephalopathy?
Nystagmus (most common ocular sign).
Ophthalmoplegia, such as lateral rectus palsy and conjugate gaze palsy.
Q: What type of neuropathy is commonly seen in Wernicke’s encephalopathy?
Peripheral sensory neuropathy is common in Wernicke’s encephalopathy.
Q: What investigation can help diagnose Wernicke’s encephalopathy?
Decreased red cell transketolase levels.
MRI of the brain may show characteristic changes.
Q: What is the treatment for Wernicke’s encephalopathy?
Urgent thiamine replacement is crucial for treatment.
Q: What syndrome can develop if Wernicke’s encephalopathy is left untreated?
Korsakoff syndrome may develop, leading to anterograde and retrograde amnesia and confabulation, in addition to the symptoms of Wernicke’s encephalopathy.
Q: What are common visual features of multiple sclerosis (MS)?
Optic neuritis (common presenting feature).
Optic atrophy.
Uhthoff’s phenomenon (worsening of vision with increased body temperature).
Internuclear ophthalmoplegia.
Q: What are the common sensory features of multiple sclerosis (MS)?
Pins and needles.
Numbness.
Trigeminal neuralgia.
Lhermitte’s syndrome (paraesthesiae in limbs on neck flexion).
Q: What are the common motor features of multiple sclerosis (MS)?
Spastic weakness (most commonly in the legs).
Q: What are the cerebellar signs seen in multiple sclerosis (MS)?
Ataxia (more common during acute relapse).
Tremor.
Q: What other features are associated with multiple sclerosis (MS)?
Urinary incontinence.
Sexual dysfunction.
Intellectual deterioration.
What is nitrogen oxide use linked to
subacute degeneration of spinal cord
‘oligoclonal bands’ in CSF
MS
When working out GCS motor component, what should you do if theres a difference in both sides eg on application of supra-orbital pressure he flexes his right arm and extends his left arm
Take the best score
Horners syndrome + arm and shoulder pain
pancoasts tumour
What area of the brain is affected in huntingtons
butterfly pattern in the caudate nucleus
What cranial nerve does bells palsy affect
7 - facial nerve
Is fluctuating level of conscience a feature of delirium, dementia or both
only delirium
what drugs should be avoided in myasthenia gravis
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
in status epilepticus, what is the management
ABC
in community - buccal midazolam/rectal diazepam
in hospital IV lorazepam, repeated once after 5-10mins
second line agent if still going levetiracetam or phenytoin or valproate
How are B12 injections given
initially every other day then every 3 months
what is the gold standard test for guillian barre
lumbar puncture
