Neurology Flashcards
Define and state causes of microcephaly
OFC > 3 standard deviations BELOW mean
TORCH infections, Foetal alcohol syndrome, Hypoxic ischaemic event at birth, genetic disorder.
Define and state causes of macrocephaly
OFC > 3 standard deviations ABOVE mean
Familial, Hydrocephalus, Neurogenetic condition
Define craniosynostosis + examples of conditions causing same
Abnormal and premature fusion of skull bones.
Crouzen, Apert, Pfeiffer syndromes
Where is CSF produced?
Chorioid plexus
Where does CSF flow after being produced?
Lateral ventricles -> 3rd and 4th ventricles -> posterior fossa -> basal cisterns. Reabsorbed through arachnoid granulations.
What are causes of hydrocephalus?
Over secretion- Choroid plexus papilloma
Intraventricular obstruction - Tumour, malformation, infalmmation
Extraventricular obstruction - Inflammation, tumour, MPS
Imparied resorption- Venus sinus compression
What are the clinical signs of ataxia?
Unsteady broad based gate
Slurred speech
Tremor
Nystagmus
When does post-varicella ataxia occur?
5-14 days after vesicles appear
What form of ataxia is assiciated with neuroblastoma?
Opsclonus myoclonus ataxia syndrome (OMAS)
acute and a/w rapid chaotic eye movements.
Chronic and non-progressive ataxia suggests what conditions?
Arnold Chiari malformations
Bandy Walker malformation
Chronic and progressive ataxia suggests what disorders?
Friedrichs ataxia
Ataxia telangectasia
Abetalipoproteinaemia
Metachromatic leukodystrophy
What are features of frontal lobe seizures?
Nocturnal, dystonic posturing, salivation
multiple per night
What are features of Temporal lobe seizures?
Aura - commonly rising epigastric sensation
triggered by fear, deja vu, jamais vu
What are features of parietal lobe seizures?
Contralteral tingling. Electric shock sensations
What are features of Occipital lobe siezures?
Contralateral visual changes -coloured lines and spots
Describe Juvenile Myoclonic epilepsy
Adolescent F >M
Brief, bilateral, arythmic jerking of limbs 1-2 hours after waking.
What EEG changes are seen in Junvenile Myoclonic Epilepsy?
3-6Hz spike or polyspike and slow wave discharge
What EEG changes are seen in Childhood epilepsy with centro-temporal spikes?
Centro-temporal spikes (shocker!)
Bilateral symmetrical 3Hz spike and wave pattern on EEG suggests what?
Absence seizure
What is the treatment for infantile spasms?
Prednisolone, Vigabatrin
Ketogenic diet maybe
What is the treatement for absence seizures?
Ethosuximide, sodium valproate
What is the treatement for Juvenile myoclonic epilepsy?
Sodium Valproate, levetiracetam, lamotrigine
What is the treatment for Lennox Gastaut Syndrome?
Sodium Valproate, Lamotrigine, clobazam
What is the treatement for benign focal epilepsies of childhood?
Levetiracetam, Sodium Valproate, Carbamazepine
What epilepsy syndromes does carbamezapine make worse?
Juvenile myoclonic epilepsy, absence epilepsy,Lennox Gastaut Syndrome
What are the side effects of Sodium valproate?
Liver dysfunction, hair loss, weight gain, thrombocytopenia, poly cystic ovaries, abnormal behavious, teratogenic
What are side effects of Levetiracetam?
Anxiety, depression, insomnia, Skin reactions
What are the side effects of Lamotrigine?
Agitation, dry mouth, insomnia, skin reactions
What are the side effects of carbamezapine?
confusion, ataxia, hyponatraemia, Leucopenia, thrombocytopenia, cutaneous drug reactions
What are the side effects of phenytoin?
GingIval hyperplasia, liver dysfunction, skin reaction. Vit D deficiency
Megaloblastic anaemia.
Arrhythmia if IV
What are the side effects of ethosuximide?
Agranulocytosis, leucopenia.
Stevens-Johnson Syndrome.
What are the side effects of Vigabatrin?
Peripheral field defects
What do infantile spasms look like?
Runs of flexion and extension spasm with high-pitched cry
Define West Syndrome and its associated EEG changes
Infantile spasm, developmental regression and interictal hypsarrhythmia on EEG
What Neurocutaneous syndrome are infantile spasm associated with?
Tuberous Sclerosis
Describe Benign familial infantile seizures
Focal seizure +/- generalisation. NOT SPASM
Resolves by one year of age
Describe childhood epilepsy with centro-temporal spikes
Usually focal, nocturnal, involving face and mouth.
Classic EEG finding
Most resolve by age 16.
Severe intractible seizure + developmental delay and regression beteween events suggests what?
Lennox-Gestaut syndrome
Aquired aphasia in previously well child + self limiting seizures suggests what?
Landau-Kleffner Syndrome
What are the diagnostic criteria of NF1?
2 or more of:
6 or more cafe au lait spots
Dermal neurofibromas
Freckles in axilla or inguinal area
Lish nodules in iris
Family history of NF!
Optic glioma
What do all NF1 patients need as part of annual review?
Opthal assessment
BP monitoring (A/w renal aretery stenosis and pheochromocytoma)
Neuro + skeletal assessment
What are the features of NF2?
Palpable inredermal schwannomas, cataracts, retinal hamartomas, mononeuropathies.
What are the derm features of Tuberous sclerosis?
Ash leaf macules, Shagreen patch, periungula fibromas (fleshy lump in nail beds)
What is tuberous sclerois associated with?
Infantile spasms,
ASD/ ID
Retinal hamartomas
Giant cell astrocyromas
PCKD
Describe Sturge Weber Syndrome
Port wine stain in distribution of trigeminal nerve
Focal epilepsy
Intellectual disability (ID)
Stroke
Describe spinal muscular atrophy
AR.
Normal tone at birth but at 6 months, hypotonic, absent reflexes, tongue fasiculations and diaphragmatic breathing
What is the treatment for Spinal muscular atrophy?
Nusinersen
Describe Duchenne muscular dystrophy
x-linked recessive
Delayed early motor milestones
calf pseudohypertrophy and Gower’s sign
Cannot walk by 12 years.
What is Gower’s sign?
Patient using arms to ‘walk up’ own body in order to compensate for hip/thigh muscle weakness
What is Becker’s muscular dystrophy? Describe its mode of inheritance.
Allelic of Duchenne. X-linked recessive
Milder from of DMD
Has upper limb involvement.
Still walking >12 years
Describe the symptoms of Friedreich’s Ataxia and it’s mode of inheritance
AR
Progressive ataxia
Posterior spinal cord dysfunction (loss vibration and proprioception)
Loss of deep tendon reflexes, lower limb weakness
Vision problems
How does CSF fluid look on T1 sequence MRI?
black
How does CSF look in T2 sequence MRI?
white
How does CSF fluid look on FLAIR sequence MRI?
Also white, but paler relative to T2
What is flair MRI used for?
investigation lesions close to ventircles
What is Magnetic Resonance Angiography used for?
intracranial vasculature. Used in conditions such as intracranial arteriovenous malformations (e.g. Sturge–Weber syndrome) and blockages or stenosis.
What is positron emission tomography?
labelled radioactive tracer is injected into the body, which gives off gamma rays that are detected by a scanner. use depends on the tracer
What is an electroencephalogram (EEG) ?
Typically, 21 numbered electrodes are placed in defined positions in each of five locations (F –frontal, C – central, P – parietal, T – temporal, O – occipital). A channel is the voltage difference measured between pairs of these electrodes.
What EEG rythm becomes predominant after 3 years of age?
Alpha
What activation proceedure is used when generating EEG to induce absence?
hyperventilating
Sleep deprived activation EEG is used to detect what types of seizure?
juvenile myoclonic epilepsy
Describe electromyography
Inserting a needle electrode directly into the muscle under investigation. The muscle action potentials are captured on recording equipment and amplified as a sound
What do Nerve injury and myopathic conditions look like on electromyography?
Fibrillation potentials and characteristic changes in EMG pattern.
How do nerve conduction studies work?
An electrode records the compound muscle action potential (CMAP) over the desired muscle group. A stimulating electrode is placed at two measured points along the nerve pathway. The latency, amplitude, wave form and velocity of conduction are all recorded. Normal age-specific values allow for comparison.
What are nerve conduction studies used for?
Investigate clinical suspicion of a peripheral neuropathy and can allow classification into axonal degeneration or demyelinating conditions
What are visual evoked potentials used to detect?
Lesions of the sensory visual pathways, e.g. demyelination of the optic nerve in optic neuritis.
Define Idiopathic intracranial hypertension (IIH) and it’s symptoms
Syndrome characterized by increased intracranial pressure (ICP) of unknown cause. headache, nausea, blurred vision, visual loss or diplopia. Papilloedema
Define trigeminal neuralgia
Sudden, sharp ‘electric shock’ pain that is severe and affects one side of the face in the distribution of the trigeminal nerve. It can occur several times per day, triggered by movement or light touch
What is treatment for trigeminal neuralgia?
carbamazepine
What is first line treatment for tonic-clonic epilepsy?
sodium valproate OR lamotrigine
What is commonest cause of epilepsy in third world?
Cysticercosis
Caused by Taenia solium parasite
How is epilepsy characterised biochemically?
Increase in glutaminergic activity and a reduction in GABA-ergic activity
How does nimodipine work, and what is it used for ?
sub arachnoid haemorrhage
prevents cerbral vasospasm
what is CSF finding of sub arachnoid haemorrhage?
xanthochromia
What is EEG finding of subacute-sclerosing panencephalitis ?
Burst suppression
Can you wean antieplieptics in jouvenile myoclonic epilepsy?
No !
Very veery high relapse rate
Insidious onset weakness + rash+ blueish discolouration around eyes +/- light sensitivity is what?!
Dermatomyositis
4-5 hertz polyspike and slow wave discharge suggests what?
juvenile myoclonic epilepsy
1-2 hertz interictal slow spike waves suggests what?
lennox-gestraut syndrome
What is MOA of levetiracetam?
Binds to SV2A proteins
What is MOA of Ethosuximide?
Blocks T type voltage gated sodium channels
