Neuro Flashcards
Which type of muscle fibre is rich in myoglobulin
Type I
What does myoglobulin do
supplies oxygen to your muscles
What are the main symptoms of myopathy
Proximal weakness
Hypotonia
Wasting (or fatty infiltration)
BUT reflexes are preserved
What would be raised on blood tests in myopathy
Creatine phosphokinase
What is a myopathic unit on a EMG?
Small, short-duration, spiky polyphasic units
What is the inheritance pattern of myotonic dystrophy type I
Autosomal dominant
What is the type of mutation in myotonic dystrophy type I
Expanded trinucleotide repeat (CTG)
Within the myotonic protein kinase gene on chromosome 19
Symptoms of myotonic dystrophy
Progressive muscle weakness, particularly in upper limbs
Myotonia
Many other symptoms (cataracts, frontal balding, dysarthria and dysphagia..)
Fatigue
Is myotonic dystrophy inheritance worse through females or males
Females, with anticipation.
What are the characteristics of metabolic myopathies
Exercise intolerance/pain/fatigue
Sometimes have periodic paralyses with high or low potassium
What is the inheritance pattern of periodic paralysis channelopathies
Autosomal dominant
What is the most common age range for inflammatory myopathies?
Middle age, classically:
Polymyositis and dermatomyositis (30/40 yr old woman)
Inclusion body myositis: over 50s male
What is the management for polymyositis and dermatomyositis
Corticosteroids +/- immunosuppression and removal of tumour if present
What is the treatment for inclusion body myositis
Supportive management (no treatment)
What is the definitive investigation for muscle disease
Muscle biopsy (determines fibre type, inflammation, dystrophic and histochemical changes)
What is the classical gait in Duchenne
Waddling gait
Where does pseudo-hypertrophy happen in Duchenne’s
Calves
What is a common cause of death in Becker’s muscular dystrophy
Cardiac arrhythmias
Characteristics of myopathies due to thyrotoxicosis
Shoulders mainly
Brisk reflexes
+/- fasciculations and atrophy
What drugs can cause myopathy
Steroids
Satins
Potassium-losing drugs
(also: chloroquine, amiodarone, doxorubicin, Zidovudine)
What type of virus is polio virus
Enterovirus
What percentage of patients with poliomyelitis have paralysis
0.1%
Symptoms of poliomyelitis
Abortive: self limiting GI and resp symptoms
Non-abortive: features of abortive with meningism. Complete recovery
Paralytic: abortive symptoms -> non-abortive -> myalgia and asymmetrical paralysis. Sometimes resp failure but usually lower limbs in children. Can cause bulbar involvement.
How is poliomyelitis distinguished from GBS
Polio doesn’t have sensory symptoms and has asymmetry
CSF findings in poliomyelitis
Same as viral meningitis
Raised protein
Increased no. lymphocytes
Normal glucose
Increased numbers of polymorphs initially
Prognosis for polio
If there is good resp support, often low mortality and good improvement in muscle power. In affected limbs in children, bone growth is retarded, resulting in a wasted, shortened limb
Which type of neurofibromatosis has peripheral predominance
Type I
Characteristics of neurofibromatosis type 1
Neurofibromas along peripheral nerves
Café-au-lait spots (>5 or >1.5cm)
Cutaneous fibromas
Scoliosis
Endocrine abnormalities
Neural tumours
Characteristics of neurofibromatosis type 2
Vestibular schwannomas (previously called acoustic neuromas)
Other intracranial and intraspinal tumours
Wernicke-Korsakoff syndrome has a deficiency of which vitamin
B1 (thiamine)
What are the two phases of Wernicke-Korsakoff’s
Acute phase: Wernicke’s encephalopathy
Chronic phase: Korsakoff’s psychosis
Triad symptoms of Wernicke’s encephalopathy
Ocular signs
Ataxia
Confusion
(e.g. nystagmus, broad-based gait, amnesia)
What symptom is classical of Korsakoff’s psychosis
Confabulation
(poor short-term memory so makes it up)
What are the symptoms of Horner’s syndrome
Unilateral Miosis (constricted pupil)
Partial ptosis
Ipsilateral anhidrosis
Causes of Horner’s syndrome
Lesion at any level of the sympathetic pathway (e.g. tumour, stroke, disease, aneurysm, dissection)
What is an acute painful Horner syndrome until proven otherwise?
Dissection of the ipsilateral internal carotid artery
What does Normal Pressure Hydrocephalus cause
Reversible dementia
Triad: Urinary incontinence, dementia and gait abnormalities
Can look like Parkinson’s but doesn’t respond to L-Dopa
Over several months
What is the management for Normal Pressure Hydrocephalus
Ventricle-peritoneal Shunt
What causes Normal Pressure Hydrocephalus
Impaired CSF reabsorption
Triggered by a head injury or bleed.
Symptoms of acute hydrocephalus
Nausea
Vomiting
Diplopia
Fluctuating conscious level
Papilloedema
Ataxia of gait
Symptoms of headache of raised ICP
Headache aggravated by: bending, coughing, straining, in the morning. Getting worse and worse
With:
Vomiting
Visual obscurations
Progressive focal neuro signs
Papilloedema
What is tonsillar herniation
When a space-occupying lesion causes the cerebellar tonics to herniate through the foramen magnum causing brainstem compression and death - ‘coning’
What are the three subtypes of cerebral palsy
Spastic (90%)
Dyskinetic
Ataxic
What percentage of cerebral palsy is due to hypoxic-ischaemic encephalopathy
10%
How does cerebral palsy present in infancy
Abnormal tone and posture
Delayed motor milestones
Feeding difficulties
What are the symptoms of anterior spinal artery syndrome
Segmental pain
Urinary retention
Flaccid -> spastic paraparesis (corticospinal)
Loss of pain and temperature sensation below lesion (spinothalamic)
areflexia -> hyperreflexia
What are the three sections of the GCS
Eye opening
Verbal responses
Motor responses
GCS: eye opening scale
1-4 AVPU
GCS: verbal response scale
1- None
2 - Incomprehensible sounds
3 - Inappropriate words
4 - Disorientated speech
5 - Orientated speech
GCS: motor response scale
1- None
2 - Extensor response to pain (Decerebrate response)
3 - Flexor response to pain (Decorticate response)
4 - Flexion/withdrawal to pain
5 - Localisation of painful stimulus
6 - Obeys commands
What is ADEM
Acute disseminated encephalomyelitis
Demyelination within the CNS
Presents following a viral infection or vaccination
Can cause encephalitis
What are the three places that oculomotor disorders can arise from
Supranuclear (cerebral hemispheres)
Internuclear (brainstem)
Infranuclear (nerves supplying muscles of the eye)
What are the features of Creutzfeldt-Jakob disease
Memory problems
Behavioural changes
Poor coordination
Visual disturbances
(rapidly progressing dementia +/- myoclonus = always assume prion disease until proven otherwise)
What is the classical CT appearance for cerebral abscess
Ring enhancing lesion
Central area of low density
Surrounding area of oedema
Which is the first line treatment for absence seizures?
Sodium valproate
What is the first line treatment for focal seizures?
Carbemazepine
What is first line treatment for Bell’s palsy?
Prednisolone
What is the first line treatment for Guillain Barré syndrome?
IVIG
What is first line treatment for Ramsey-Hunt syndrome?
Prednisolone and acyclovir
What is Ramsey-Hunt syndrome?
Shingles in the facial nerve
In what order does diabetic polyneuropathy happen? (the different sensory modalities)
First numbness and tingling
Then proprioceptive balance issues
Then motor function
What can Vit B12 deficiency cause?
Peripheral neuropathy
Subacute combined degeneration of the cord
Optic neuropathy
Dementia/encephalopathy
What can unusually cause Horner’s syndrome?
T1 nerve root involvement
What is a Pancoast tumour
Squamous cell lung carcinoma at the apex of the lung - often causes brachial neuropathy
How would you treat fatigue as a symptom of MS?
Modafinil or Amantadine
How could you treat tremor in MS?
Clonazepam
How could treat pain symptoms in MS?
Amitryptiline
How could you treat spasticity in MS?
Baclofen
What is Meralgia Paraesthetica
Compression of the lateral cutaneous nerve of the thigh under the inguinal ligament
What are the symptoms of Meralgia Paraesthetica
Shooting pains on the outside thigh
Numbness and painful tingling over area, highly sensitive to light touch and heat
What is Lhermitte phenomenon in MS?
Shooting pains along the limbs following flexion of the neck
What does the segmental medullary artery supply?
Branches of the cervical parts of the vertebral arteries. Supply the surface and the inside of the spinal canal at each segmental level
How many anterior and posterior spinal arteries?
One anterior, one posterior
What is Jacksonian march?
Twitching starts in the limb then ascends to trunk and then to arm (part of epilepsy)
What does spinal MND look like?
Mixed picture with UMN and LMN signs
What does primary lateral sclerosis MND look like?
Mostly UMN signs
What does progressive muscular atrophy MND look like?
Mostly LMN signs
In any major bleed with Warfarin, what do you give for management
Stop warfarin
Vitamin K 5mg IV
Prothrombin Complex concentrate
What are the classic findings on an MRI head and spine for MS?
Juxtacortical and paraventricular lesions as well as spinal cord lesions
What is autonomic neuropathy a common complication of?
Poorly controlled diabetes
Symptoms e.g. gastroparesis, postural hypotension
What is Charcot Marie Tooth?
Hereditary motor and sensory peripheral neuropathy
What is the most common genetic mutation Charcot Marie Tooth?
Mutation in the peripheral myelin protein 22 (PMP22) gene
What is the different between Charcot Marie Tooth Type 1 and Type 2?
Type 1 is demyelinating (PMP22)
Type 2 is axonal
What are the key clinical features of Charcot Marie Tooth?
Thickening and enlargement of the nerves themselves
Symmetrical distal muscular atrophy (champagne bottle legs, claw hands)
Pes cavus (arched feet)
How is Charcot Marie Tooth diagnosed?
Nerve conduction studies
Genetic testing
Patients with type one have reduced conduction velocity whereas this is normal in type 2
What is the medial longitudinal fasciculus?
A brainstem structure connecting the 3rd and 6th cranial nerve nuclei on opposite sides of the brainstem to coordinate horizontal conjugate gaze (abduction of one eye with the adduction of the other)
With internuclear ophthalmoplegia, is the lesion ipsilateral or contralateral to the eye that can’t adduct?
Ipsilateral
How do you diagnose Creutzfeldt-Jakob disease?
Tonsil biopsy (used to be brain biopsy but now know that there are extra neural sites - tonsils and olfactory mucosa)
How does Cretuzfeldt-Jakob disease present?
Rapidly progressive dementia, psychiatric impairment, myoclonus
What does an EEG show in Creutzfeldt-Jakob disease?
Periodic sharp-wave complexes
What is the difference between sporadic and variant Creutzfelt Jakob disease on MRI?
Sporadic - high signals in the posterior thalamus
Variant - high intensity in the caudate and putamen (basal ganglia)
What is Mannitol?
Osmotic diuretic used to reduce intracranial pressure
What is Acetazolamide used for?
Treats idiopathic intracranial hypertension
What are the four Parkinson-plus syndromes?
Progressive supra nuclear palsy
Multiple system atrophy
Cortico-basal degeneration
Lewy body dementia
How does progressive supranuclear palsy present?
Parkinsonism and vertical gaze palsy
How does multiple system atrophy present?
Parkinsonism and early autonomic features: postural hypotension, incontinence, impotence
How does cortico-basal degeneration present?
Parkinsonism and spontaneous activity involving the affected limb or akinetic rigidity of that limb
How does Lewy body dementia present?
Parkinsonism and fluctuations in cognitive impairment and visual hallucinations, often before Parkinsonian features develop
What are the five main ways that MS presents?
- Sensory disease (patchy paraesthesia)
- Optic neuritis (loss of central vision and painful eye movements)
- Internuclear ophthalmoplegia (a lesion in the medial longitudinal fasciculus of the brainstem)
- Subacute cerebellar ataxia
- Spastic paraparesis (transverse myelitis, including Lhermitte’s sign).
What are oligoclonal bands in the CSF indicative of?
Autoimmune processes in the CSF
MS
Lyme disease
SLE
Neurosarcoid
What are the initial symptoms of Lyme disease?
Fever, rigours, migratory polyarthritis , myalgia,
Characteristic erythema migraines rash with central clearing
What are later complications of Lyme disease?
Large joint monoarthritis
Unilateral or bilateral facial nerve palsy
Neuropathic pain
Palpitations
Which drugs can worsen the symptoms of myasthenia gravis?
Beta blockers
several antibiotics and antimalarials
Lithium and other drugs used in psychiatry
How do you clinically distinguish encephalitis from meningitis
Encephalitis has altered mental state whereas meningitis does not
What is Miller-Fisher syndrome?
Similar to GBS (follows a viral infection) but starts in the eyes not peripherally
What is the triad in Miller-Fisher syndrome?
Ataxia, areflexia and ophthalmoplegia
Interferon beta is used to treat what condition?
Relapsing and remitting MS
Myasthenia gravis is associated with what condition in the chest?
Thymoma
How is diazepam administered?
Rectally
How is midazolam administered?
Buccally
How is lorazepam administered?
IV
In the case of a suspected subarachnoid haemorrhage but a negative cT, what is the next best investigation to do?
Lumbar puncture (as long as there is no raised ICP)
This is performed 12 hrs after the onset of the headache to look for xanthochromia
Why can Isoniazid (TB treatment) cause polyneuropathy?
Isoniazid causes B6 deficiency which can cause polyneuropathy
When is a stroke classified as a TACI (total anterior cerebral infarct) - Bamford/Oxford classification
Contralateral hemiparesis or hemiplegia AND
Contralateral homonymous hemianopia AND
Higher cerebral dysfunction
When is a stroke classified as a lacunar infarct? LACI
Pure motor stroke, pure sensory stroke, sensorimotor stroke, or ataxic hemiparesis
When is a stroke classified as a PACI?
2 of the following, or higher cerebral dysfunction alone:
- Contralateral hemiparesis or hemiplegia AND
Contralateral homonymous hemianopia AND
Higher cerebral dysfunction
What arteries are affected in a tACI?
Anterior AND Middle cerebral arteries
What artery is affected in a PACI
Anterior OR Middle cerebral arteries
When is a stroke classified as a POCI
Cerebellar dysfunction, OR
Conjugate eye movement disorder, OR
Bilateral motor/sensory deficit, OR
Ipsilateral cranial nerve palsy with contralateral motor/sensory deficit, OR
Cortical blindness/isolated hemianopia.
What arteries are affected in a POCI?
Vertebrobasilar arteries and the associated branches
Which condition causes multiple ring enhancing lesions on MRI?
Toxoplasmosis
What are periventricular ring enhancing lesions on MRI indicative of?
Primary cerebral lymphoma, caused by Epstein-Barr virus
What nerve is likely to be damaged in fracture of the fibular head (e.g. in a head on collision, car into pedestrian)?
Common fibular nerve (common perineal nerve) - wraps round the head of the fibula
Causes foot drop because it innervates tibias anterior muscle which dorsiflexes the ankle
Positive Trendelenbur’s test, weak abduction in the affected hip is indicative of injury to which nerve?
Superior gluteal nerve
What is the path of the tibial nerve?
Branches off the sciatic nerve, follows the popliteal vessels, crossing to the medial side and running down to the posteriomedial side of the ankle.
Then divides into the medial and lateral plantar nerves to innervate the flexors and small muscles of the foot
How would obturator nerve injury present?
Medial thigh or groin pain
Weakness with leg adduction
Sensory loss in the medial thigh of the affected side
Are extradural haemotoma source of bleeding arterial or venous?
Middle meningeal artery
Are subdural haemotoma source of bleeding arterial or venous?
Venous
Acute cord compression in the setting of anticoagulation and possible iatrogenic dural puncture (ie.e epidural) is suspicious of what?
Epidural haemotoma
After a one-off seizure (first seizure), how long can you not drive for?
6 months
What is apraxia
The inability to visualise movement (e.g. follow instructions on movement)
What causes apraxia?
Corticobasal degeneration
What are the symptoms of corticobasal degeneration
Apraxia
Parkinsonian features
Alien limb phenomenon
What is the first line management for myaesthenia gravis
Pyridostigmine
How does Pyridostigmine work?
Cholinesterase inhibitor
What is first line management for a subdural haemotoma?
Burr hole craniotomy
What is the commonest cause of an intracerebral haemorrhage?
Hypertension
What is benign positional paroxysmal vertigo?
The presence of debris in the semicircular canals causing vertigo on head movement
How do you treat BPPV (benign positional paroxysmal vertigo)?
Epley manoeuvres
What can present with nocturnal headaches that occur around the same eye and are accompanied by nausea, watering of the eye, redness of the eye?
Acute angle glaucoma
Cluster headaches
What is used for prophylaxis in cluster headaches?
Verapamil
What is the underlying pathology in primary lateral sclerosis MND?
Degeneration of the Betz cells in the motor cortex
In sternocleidomastoid neuropathology, weakness in the sternocleidomastoid leads to weakness turning the head in the same or opposite direction?
Opposite direction
What is Nimodipine?
Calcium receptor antagonist
What does Nimodipine do?
Acts in the cerebral vasculature to decrease risk of vasospasm in patients with subarachnoid haemorrhage
Hoe does autoimmune encephalitis present in it’s earliest stage?
Fever, headaches, diarrhoea and upper respiratory tract infection
What is the anti-epileptic of choice in women of reproductive age with tonic-clonic seizures?
Lamotrigine
What is the management for haemorrhagic strokes?
Reversal of anticoagulants (beriplex/octaplex/vit K) and aggressive BP control
Systolic blood pressure should be kept <140mmHg within an hour of admission and ideally above 120mmHg. Use of GTN or labetalol for this
How do cluster headaches normally present?
Short lasting, frequent, perio-orbital, sometimes with additional features such as lacrimation
Why is recommended that women with epilepsy breastfeed after giving birth to their child?
So that the baby can withdraw from the medication slowly
What is mononeuritis multiplex?
A type of peripheral neuropathy, where multiple separate nerves start to be affected, either at the swim time or sequentially,
Affects both sensory and motor
What are risk factors for mono neuritis multiplex?
Diabetes
Vasculitis
Immune diseases
Infections
Sarcoidosis
Rarely, certain jelly fish stings
What nerve innervates the triceps?
C7-8
What can cause transient proteinuria?
Seizures
Strong infections
Pregnancy
Heavy exercise
How is respiratory function monitored in GBS?
FVC
What are the features of a medical third nerve palsy?
Double vision
Dropping of eyelid
Eye deviated inferiorly and laterally
What is a common cause of medical third nerve palsy?
Diabetes
What are the features of a surgical third nerve palsy?
Painful and dilated pupil
What is Weber’s syndrome
Midbrain stroke
What are the features of Weber’s syndrome?
Ipsilateral third nerve palsy
Contralateral hemiparesis
What causes the triad of ‘wet, wobbly and weird’? (urinary incontinence, poor mobility, not himself)
Normal pressure hydrocephalus
What would you see on CT head if someone had Normal pressuer hydrocephalus?
enlarged ventricles and absent sulci
What is first line drug management for Alzheimer’s?
Donezapil
(Memantine is second line)
What class of drug is Donezapil?
Acetylcholinesterase inhibitor
What does GBS usually show on LP?
Isolated rise in protein
What can cause intermittent dizzy spells
BPPV
Vestibular neuritis - this often follows and upper resp infection
What is Cushing’s reflex?
When ICP rises, sometimes it activates both the sympathetic and the parasympathetic systems
Increased BP, decreased HR, irregular breathing
What type of tremor is autosomal dominant and often increases during times of anxiety or stress?
Essential tremor
What is the management of status epilepticus?
2 attempts at using benzodiazepines to stop
If unsuccessful - IV Phenytoin
What can Phenytoin cause/what do you need to monitor?
Can cause bradycardia and hypotension - monitor ECG and BP
What does the DDANISH or cerebellar dysfunction stand for?
Dysdiadochokinesia
Decomposition of movement
Ataxia
Nystagmus
Intention tremor
Scanning speech/staccato
Hypotonia
What neurological condition can b12 deficiency cause?
Subacute combined degeneration of the spinal cord
Polyneuropathy, sensory ataxia,
e.g. after a terminal ileum resection for Crohns
What is the gold standard investigation in investigating vascular abnormalities in the brain (e.g. after a subarachnoid haemorrhage)?
Digital subtraction catheter angiography (DSA)
What is Klumpke’s palsy?
Damage to the lower brachial plexus
Small hand muscle paralysis
Ptosis
Dermatomal sensory disturbance
What is the earliest that you can do an LP in suspected SAH?
12 hours (sufficient time for RBC breakdown to form xanthochromia)
Is a contrast or non-contrast CT better for SAH?
Non-contrast
What is the management of idiopathic intracranial haemorrhage?
Weight loss and close monitoring
What is second line management of idiopathic intracranial haemorrhage?
Acetazolamide
What is hepatitis C related cryoglobulinaemia?
Cryoglbulinaemia are proteins that become insoluble at reduced temperatures. This causes a vasculitic syndrome including neuropathy.
What is mononeuritis multiplex?
Sub-acute painful multifocal neuropathy
What does vasculitic neuropathy look like?
Mononeuritis multiplex and systemic inflammation
Generalised tonic-clonic seizure after head trauma with rapid recovery and no post-ictal confusion is consistent with what?
Reflex anoxic seizure - caused by overactivity of the vagus nerve, causing vasodilation and collapse froma temporary reduction in cerebral perfusion
What does a hyper-intense lesion indicate in a stroke?
Haemorrhage not infarct
What is an important side effect of Ropinirole (used in Parkinson’s) that you need to monitor for?
Impulsivity - pathological gambling, hypersexuality
How does Friedrich’s ataxia present?
Frequent falls and lower limb weakness and gait abnormalities
Signs on examination: cerebellar and mixed UMN and LMN signs
Involvement of the dorsal columns can lead to impaired proprioception and vibration sense
Other clues: high-arched palate, pes cavus, kyphoscoliosis
Non motor features: hypertrophic obstructive cardiomyopathy, reduced visual acuity, T1DM, deafness
What neuropsychological finding is most likely to suggest a right parietal lesion in a right-handed woman?
Visual inattention/neglect
When should Sumatriptan be taken?
Once the headache starts but not during the aura phase
When is a decompressive hemicraniectomy considered in stroke?
In patients less than 60 years old who have severe stroke symptoms, reduced consciousness and an infarct of at least 50% of the MCA territory
What is subacute combined degeneration of the cord caused by?
B12 deficiency
What does pyridoxine (B6) deficiency cause?
Sensory neuropathy - can be induced by Isoniazid s it is now always co-prescribed with Pyridoxine
What are the classic signs of internuclear opthalmoplegia?
Failure to adduct on the affected side and nystagmus on the contralateral side
What is the first-line treatment for trigeminal neuralgia?
Carbemazepine
What is the first line treatment for an acute cluster headache?
Nasal triptan
What is the CSF profile in GBS?
Raised protein with a normal white cell count
What are the features of Wilson disease?
Cerebellar signs, tremor and rhythm abnormalities in a young patient
What is the inheritance pattern in Wilsons disease?
Autosomal recessive
Thrombectomys are best for thrombus where?
Proximal middle cerebral artery or internal carotid artery
Which opiate can cause seizures?
Tramadol (lowers seizue threshold). Can also cause delirium and impotence
What is the clinical picture of cervical spondylosis?
UMN signs in lower limbs
LMN in upper limbs
Pain on neck flexion (Lhermitte’s sign)
A frontal mass on CT that crosses the midline in a teenager is most likely to be what type of tumour?
Grade 4 astrocytoma, known as glioblastoma multiforme
What type of ataxia does a positive Romberg’s sign indicate?
Sensory ataxia rather than cerebellar ataxia
How does Brown Sequard Syndrome present?
Ipsilateral paralysis, loss of vibration and position sense and hyperreflexia below the level of the lesion
Contralateral loss of pain and temperature sensation usually beginning about two to three segments below the level of the lesion
What would you see on a CT in someone who had a cerebral abscess?
Ring enhancing lesion
Why do you get homonymous hemianopsia wiht macular sparing when there is a bleed on just one side of the brain?
Because the macular is supplied by both sides of the brain
What is the first line treatment for Wilson’s disease?
Penicillamine
What are the potential risks of using Penicillamine?
Drug induced lupus
Drug induced myaesthenia gravis
What is diabetic amyotrophy?
Asymmetric muscle wasting often seen in the thighs
What does a ‘Lentiform-shaped heterogenous hyper-dense extra-axial collection adjacent to the left squamous temporal bone’ suggest?
Extra-dural haemmorrhage
Why is hyperventilation an important part of the management of a raised ICP?
To keep the CO2 levels within normal limits
High carbon dioxide levels cause the cerebral vessel to dilate, increasing the blood flow to the brain and increasing the ICP further
