Neurology Flashcards
Ataxia telangiectasia (inheritance)
Inheritance pattern
Autosomal recessive
Friedrich’s ataxia (inheritance)
Autosomal recessive
Lateral medullary syndrome
Wallenberg’s syndrome
Occlusion of posterior inferior cerebellar artery (PICA)
-> ataxia, nystagmus, ipsilateral Horner’s/pain and temp sensation loss, contralateral limb sensory loss
Right pupil larger than left, no reaction to light directly or consensually
Reduced/absent deep tendon reflexes
Adie’s pupil
Afferent pupillary defect
Findings
Due to impaired ipsilateral optic nerve function
Brief paradoxical pupillary dilatation on swinging light test
Bilaterally small pupils
Accommodate but do not react to light
Argyll Robertson pupils
Ptosis
Ipsilateral small pupil
Syndrome?
Horner’s syndrome
Ipsilateral ptosis
Pupil enlargement
Third nerve palsy
Miller Fisher Syndrome
Features
Antibodies
Variant GBS
Triad: ataxia, external ophthalmoplegia, areflexia
GQ1B antibodies
Gerstmann’s syndrome
Dominant parietal lobe infarct
Features
Finger agnosia
Acalculia
Agraphia
Left-right limb disorientation
Contralateral hemiparesis and sensory loss; worse in lower limbs
vessel?
Anterior cerebral artery
Contralateral hemiparesis; upper limbs affected more than lower
Contralateral homonymous hemianopia
Aphasia
Middle cerebral artery
Contralateral homonymous hemianopia with macular sparing
vessel?
Posterior cerebral artery
Weber’s syndrome (branches of posterior cerebral artery that supply midbrain)
Signs
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
Lateral pontine syndrome (anterior inferior cerebellar artery)
Ipsilateral: facial paralysis and deafness
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
Retinal/ophthalmic artery
Presentation
Amaurosis fugax
Mild-mod learning disability
Narrow face, large ears, flexible fingers, large testicles
Condition?
Gene
Inheritance
Fragile X syndrome
FMR-1 gene
X linked dominant
Torticollis muscle
Ipsilateral sternocleidomastoid
Fixed gaze palsy
Eyes look towards the lesion e.g. fixed gaze lesion to the right = right sided lesion
Multi-system atrophy
Parkinsonism
Autonomic disturbance: erectile dysfunction, postural hypotension, atonic bladder
Cerebellar signs (ataxic gait)
Progressive supranuclear palsy
Vertical gaze
Postural instability
Parkinsonism
Cognitive impairment within months
Dementia with Lewy bodies
Visual hallucinations
Normal pressure hydrocephalus
Dementia + urinary incontinence
Subacute combined degeneration of the spinal cord (Vitamin B12 and E deficiency)
Affects posterior columns
- Ipsilateral UMN weakness (spastic paresis)
- Ipsilateral loss of proprioception and vibration
Ataxic gait
Syringomelia features
LMN signs and spinothalamic loss (pinprick and pain sensation)
Neurocutaneous disorders
Retinal hamartomas
Epilepsy
Developmental problems
Ash leaf spots
Shagreen patches
Subungal fibromata
Condition?
Tuberous sclerosis
Cafe au lait spots
Axillary/groin freckles
Neurocutaneous disorders
Iris hamartomas
NF2: bilateral acoustic neuromas and other CNS tumours
Neurofibromastosis 1 and 2
Pituitary apoplexy
Bleeding into/impaired blood supply of pituitary
Sudden onset headache and non-functioning pituitary
Bilateral spastic paresis and loss of temp/temp sensation
anterior spinal artery occlusion
Sensory loss of postero-lateral aspect of leg and lateral aspect of foot, weakness in plantar flexion of foot, reduced ankle reflex, positive sciatic nerve stretch test
S1
Ramsay Hunt treatment
oral aciclovir and prednisolone
CT head: hypodense (dark), crescentic collection around the convexity of the brain
diagnosis?
chronic subdural haematoma
Restless legs treatment
Dopamine agonist - Pramipexole, ropinirole
Cerebellar ataxia and telangiectasia, onset is in childhood
Immunodeficiency
Ataxic telangiectasia
Infantile-onset spinocerebellar ataxia
Friedreich’s ataxia
Causes of facial palsy
- Bells palsy
- Ramsay-Hunt syndrome
- acoustic neuroma
- parotid cancer
- HIV
- multiple sclerosis
- diabetes mellitus
Distal muscle wasting and weakness
Charcot-Marie Tooth
Bilateral spastic paresis and loss of pain/temperature sensation
occlusion where
Anterior spinal artery occlusion
Dorsal columns no affected as these are posterior
Lambert Eaton syndrome
Which channels affected
Voltage gated calcium channels
Myasthenia Gravis
Channels affected
Nicotinic acetylcholine receptors
Treatment of myasthenic crisis
IV immunoglobulins OR
plasma exchange
Third nerve palsy and headache
Important to exclude…
Posterior communicating artery aneurysm
Treatment of SAH
Calcium channel blocker (Nimodipine) -> prevents vasospasm
Measure of disability or dependence in activities of daily living in stroke patients
Barthel index
involuntary, sudden, jerking movements which occur contralateral to the side of the lesion
Hemiballism
