General Neurology Flashcards
3 most common causes of ataxia?
Acute cerebellar ataxia
Drug intoxication
Guillain-Barre Syndrome
2yo child presents with sudden inability to sit/walk with wide based gait. Coordination impaired. Viral illness 2/53 ago. Cause of ataxia?
Acute cerebellar ataxia
Treatment of acute cerebellar ataxia?
Supportive, usually improved within 2-3 weeks however can last up to 3 months
Can sometimes give steroids/IVIG
Most common degenerative ataxia?
Ataxia Telangiectasia
18mo child now walking with narrow based gait, nystagmus and apraxia of horizontal gaze. Recurrent sinopulmonary infections. Telangiectasias in conjunctivae and on bridge of nose. High AFP. What dx?
Ataxia Telangiectasia
Immune deficiency seen in Ataxia Telangiectasia?
Decreased secretory IgA
Diminished IgG2, Ig4 and IgE
What malignancy seen in ataxia telangiectasia and what RF to avoid?
Lymphoreticular tumours, brain tumours
Highly radiation sensitive (avoid unnecessary imaging)
Most common hereditary ataxia?
Friedrich’s Ataxia
Gene mutation and inheritance of Friederich’s Ataxia?
AR
Trinucleotide repeat expansion GAA causing mutation in mitochondrial frataxin protein
11yo child presents with ataxia, loss of lower limb reflexes and diminished sense of vibration in lower limbs. Dysarthric speech. Might have skeletal abnormalities or hypertrophic cardiomyopathy. What dx?
Friederich’s Ataxia
Management of Frederich’s Ataxia?
Antioxidant therapy - coenzyme Q10
Vitamin E
7yo boy presenting with fever, encephalopathy, ataxia, language disturbance. Recent ED presentation with enterovirus.
MRI shows hyper-intense white matter abnormalities - mostly subcortical
CSF - increased protein
What dx?
Acute Disseminated Encephalomyelitis (ADEM)
Management of ADEM?
?no management
Steroids - IV methylpred then a PO pred slow wean
IVIG if not responsive to steroids
Plasmapheresis if not responsive to either
Can consider rituximab, cyclophosphamide
Risk of MS after ADEM?
2-10%
60-90% of children make full recovery without sequelae
Child presents with bilateral leg weakness, hyperreflexia and clonus, urinary retention.
MRI shows demyelinating lesions of spinal cord with cord swelling and enhancement. Dx?
Transverse myelitis
Treatment of transverse myelitis?
IV steroids with a slow PO steroid wean
IDC if urinary retention
Plasma exchange if not responsive to steroids
Child presents with vision worsening over the last day or so, on testing has central scotoma and failed Ishihara tests with red numbers. Eye pain with eye movement. Had a viral illness 1/52 ago. Dx?
Optic neuritis
Optic neuritis treatment?
Steroids (MRI B should be completed prior to commencing)
Two arterial circulations in the brain and the point at which they meet?
Anterior internal carotid
Vertebrobasilar
Meet at Circle of Willis
Risk factors for arterial ischaemic stroke?
Arteriopathy (50%) - moyamoya
Cardiac (25%) - inc PFO, post cath/surg, arryhtmia/endocarditis
Haematological - sickle, prothrombotic diseases
Child presents with headache, diplopia, lethargy, then has a seizure in ED. Reading through their history you see they have a promthrombotic condition/maybe they have leukaemia and chemo regimen includes L-aparaginase. What dx?
Cerebral Venous SInus Thrombosis (CVST)
Risk factors for CVST?
Pro-thrombotic conditions - V Leiden, protein C/S deficiency, anti phospholipid syndrome Dehydration Iron def anaemia Drugs - L-asparaginse, COCP Sepsis/DIC Local infection - otitis media, mastoiditis Trauma Venous malformation
Ix for CVST?
CT or MR venography
Mx for CVST?
Heparin or enoxaparin, usually for 3 months with re-imaging for assessment.
30% have thrombus extension in first week
Regular ophthal exam for optic neuropathy secondary to raised ICP
Epidural haematoma - what vessel injured?
Middle meningeal
Subdural hameatoma - what vessel injured?
Bridging vein
Subdural haematomas of variable age?
NAI
Child presents with focal neurological deficit and psych sx, angiogram demonstrates features of vasculitis. Dx?
CNS vasculitis
- Childhood primary angitis of the CNS (cPACNS)
Secondary CNS vasculitis
Bloods for Ix of CNS vasculitis?
Inflam markers - CRP ESR FBE
Endothelial markers - vWF proposed biomarker of vasculitis (correlates w disease activity)
Bacterial cultures/viral serology
Complement/autoantibodies
Pro-coagulant profile
LP - CSF and opening pressures, neuronal antibodies
Treatment of CPACNS?
Corticosteroids (IV pulse therapy)
Antithrombotic therapy - esp if large/medium vessel involvement
Child presents with headache, seizures. MRI demonstrates increased signal intensity of occipital lobe on T2 images. No hypertension. What dx?
Posterior reversible leukoencephalopathy syndrome (PRES)
Child severely encephalopathic. Presented to ED 1 week ago and resp swab confirmed influenza. Bloods largely unremarkable however liver enzymes raised with normal ammonia level. MRIB shows symmetrical lesions in the thalami. What dx?
Acute necrotising encephalopathy
Often triggered by viral illness (HHV6, influenza) in genetically susceptible people
RANBP2 gene
Treatment of acute necrotising encephalopathy?
Steroids & IVIG
NMDA encephalitis mechanism?
IgG antibodies against NR1 subunit of NMDA receptor causing disruption of function to GABAergic neurons.
NMDA encephalitis associated with what underlying disease?
Ovarian teratoma
LP findings in autoimmune encephalitis?
Increased protein, lymphocytic pleocytosis
Opsoclonus-myoclonus associated with what underlying disease?
Neuroblastoma
CSF is produced where?
Choroid plexus
CSF flow pattern?
Lateral ventricles
- > through foramen of munro to 3rd ventricle
- > via Silvian fissure to 4th ventricle
- > via lateral foramina to basal cistern
CSF resorbed by?
Mostly arachnid villi
Some lymphatic channels & choroid plexus
Acute migraine management?
Paracetamol, environmental change Aspirin 1g (if >12yrs) Sumitriptan IN (if >12yrs) Chlorpromazine in 1L NS
Migraine prophylaxis indications?
Frequent or long lasting migraines, causing sig disability for diminished QoL
Contraindication to acute therapies
Risk of medication overuse
Menstrual migraine
Prophylaxis for migraine?
Flunarizine (Ca ch blocker)
Beta blockers (contraindicated in asthma/depression)
Cryohepatidine (antihistamine/serotonin antagonist, anticholinergic and blocks Ca channels)
Amitriptyline
Child presents with off balance, reaching for furnitures. Might be sweaty and vomit during episode. Episode lasts about 1min then resolves, usually will happen intermittently for days/week then go a longer period with out any episodes. Fhx migraine. What dx?
BPPV
