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 are the key differences between DM1 and DM2?
