Neurology Flashcards
What age group is affected by febrile convulsions?
6mo-6yr
What is the criteria for a seizure to be Simple Febrile seizure
- 95%
Brief repeated symmetric contractions of neck, trunk, extremities (flexion and extension) lasting 10-30s
Infantile Spasm
What is West Syndrome?
Infantile spasm, Developmental regression, hypsarhythmia on EEG
What age group is affected by infantile spasm?
4-8mo
80% of infantile spasms are due to ________ abnormalities, _____ or are associated with_____
80% due to metabolic or developmental abnormalities, encephalopathies or are associated with neurocutaneous syndromes (
What is seen on a Typical EEG: hypsarrhytmia
High voltage slow waves, spikes and polyspikes, background disorganization
Management of infantile spasms:
ACTH, benzodiazepines, Corticosteroids (for an acute period of time), vigabatrin (if diagnosed dura sclerosis)
Centrotemporal sharp waves on EEG?
Benign Focal epilepsy (rolandic spikes)
Focal motor seizures involving tongue, mouth, face, upper extremity usually occurring in sleep-wake transition states
Benign Rolandic Epilepsy
What age group affected by Benign Rolandic Epilepsy?
5-10 years of age - remit spontaneously in adolescence
Manage Benign Rolandic epilepsy with _______
Carbamazepine,
What age group affected by Childhood absence seizures?
6-7 years and usually grow out of it by teenage years
What medication is used for Childhood absence seizures?
and if there’s also generalised seizures?
Ethosuximide for just absent
Valproic acid (if also generalized)
Typical EEG: 3Hz spike and wave
Childhood absence seizures
Normal EEG in between seizures
Multiple daily absence seizures lasting
Childhood absence seizures
Myoclonus particularly in morning; frequently presents as generalized tonic-clonic seizures
Juvenile Myoclonic Epilepsy (Janz Syndrome)
- Can also have absence seizures
What age group is affected by Janz Syndrome
12-16year olds, autosomal dominance with variable penentrance
What would exacerbate Janz Syndrome?
Sleep deprivation, Illness, photic stimulation
Triad for Lennox-Gastraut Syndrome?
- Multiple seizure types
- Diffuse congnitive dysfunction
- Slow generalised spike and slow EEG wave
Causes for Lennox Gastraut syndrome?
Seen with underlying encephalopathy and brain malformations
Management of Janz Syndrome?
Life long treatment - Valproic acid most commonly.
Mx of Lennox-Gastraut syndrome?
Valproic acid, benzodiazepines and ketogenic diet; however, response often poor
infantile acquired aphasia post seizure?
Landau Kleffner Syndrome:
Steven Johnson syndrome associated with ______ especially with given with carbamazepine
Lamatrogine
Why do you get non-communicating hydrocephalus?
Because of failure to communicate within the brain?
“Sun setting sign” - eye gaze downwards?
Hydrocephalus
Adenoma sebaceum is related to ______
Tuberous sclerosis
What is a port wine stain related to?
Sturge Weber disease
NF 1 is more common/uncommon?
Autosomal dominant/recessive
More common - dominant
Bilateral acoustic neuroma is related to _____
Neurofibromatosis 2
Small angiokeratoms seen on face - often in malar distribution are called adenoma sebaceum. This is related to _____
Tuberous sclerosis
A child with hypopigmentation (ash leaf spots) and isolated raised plaque over lower black (Shrageen patch)
Tuberous Sclerosis
Complications of Tuberous Sclerosis?
Epilepsy (generally presented within 1 st of life)
- generally present with Infantile spasms
How do you manage infantile spasms?
ACTH, Vigabatrin
Port wine nevus syndrome in V1 distribution with associated ________-
Angiomatous Malformation _ sturge weber syndrome
Cherry red spot, hypotonia, developmental regression in infancy, death 2-5 years. Jewish!
Tay Sach’s disease
Hypersplenism (Splenomegaly), bone marrow suppression, neurological degeneration, seizures
Gaucher disease
Delayed response to previous measles infection…
Subacute sclerosing panencephalitis (SSPE)
Ddx for seiures?
Breath holding attacks
Reflex anoxic seizures (head trauma, cold food, frightened)
Bright eye appearance - and severe hypotonia (frog like posture)
SMA Type I
Waddling gait, pseudohypertrophy of the calf, run flower than their peers, one step at a time
Duchene dystrophy
Gower’s signs - what is is and what is it related to?
It’s a weird way that kids with peripheral weakness get up and it is related to Duchene Dystrophy
Temporal lobe seizures are partial seizures with ______ aura
Sensory Aura
Infantile spasms can look like _____
colic pain.
Infantile spasms with hypsarrythmia and developmental regression point to
West Syndrome
In infantile spasms CT demonstrates diffuse or localised brain disease in 70% which can be due to _______
Tuberous Sclerosis
Most common cause of Cerebral palsy?
Antenatal causes: Cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
Which one of the following does not cross suture lines?
Cephalohaematoma or Caput?
Cephalohaematoma and it develops after birth
A common complication of Cephalohaematoma?
Jaundice may develop
A 4-week-old child is brought to clinic with a red rash on her scalp associated with yellow flakes. What is the most likely diagnosis?
Seborrhoeic dermatitis
Which diet is helpful in epilepsy?
Ketogenic diet
Which seizure syndrome?
1-5 year old affected, atypical absences, falls and jerks ; EED shows slow spikes…
Lenox-Gastruat Syndrome
Teenage girl, with infrequent generalised seizure in the morning, with daytime absences and sudden shock like myoclonic seizure…
Janz Syndrome - Juvenile Myoclonic Epilepsy
Which anti epileptic has a good response to Juvenile Myoclonic Epilepsy?
Sodium Valproate
Which anti epileptic has the following side effects?
Alopecia, Ataxia, tremor, hepatitis, pancreatitis, thrombocytopaenia,
Sodium Valproate
Which anti-epileptic has the following side effects?
Dizziness and ataxia, leukopenia with agranulocytosis, syndrome of inappropriate ADH secretions, and visual disturbances (Diplopia)
Lamotrigine
Which anti-epileptic has the following side effects?
Gingival hyperplasia, Hirsutism, coarsening of facial features, megaloblastic anaemia, peripheral neuropathy, enhanced Vitamin D metabolism causing osteomalacia, Lymphadenopathy
Phenytoin
Which anti-epileptic has the following side effects?
Sore throat, fatigue, ulcers in the mouth - reduced ability to eat or drink. “Toxic epidermal necrolysis”
Stevens Johnson Syndrome
A mother brings her 2-week-old baby girl into the surgery for review. She has noted a bright red, well-circumscribed and lobulated lesion developing on her right temple. This wasn’t noted at birth but is now 5 mm in diameter… What is it?
Strawberry Naevus - not present at birth.
If a Strawberry naevus is causing visual field obstruction, how would you treat it?
Propanolol
3-5 year old with multiple seizure types, diffuse cognitive dysfunction and slow generalised spike and slow wave EEG
Lennox gastruat syndrome
How do you manage benign focal epilepsy of childhood with Rolandic centerotemporal spikes?
Carbamazapine
Seizure leads to disruption of talking –> Infantile acquired aphasia
Laundau Kleffner syyndrome
Atresia of foramine of Magendie and Luschka, resulting in complete or incomplete agenesis of the cerebellar vermis with widely separated, hypoplastic cerebellar hemispheres.
Dandy walker malformation
In Dandy Walker malformation you get two widely separated cerebral hemispheres and can also get _______ formation
Posterior cyst formation
________ is responsible for 2-4% of paediatric hydrocephalus
Dandy Walker malformation
Average age of type I Chairi malformation presentation is ____
15 yo
In Chiari Malformation type I the ________ _____ lie below the level of the foramen magnum
Cerebellar tonsils
3 common presentations of Chiari malformations Type I?
Central chord syndrome
Syringiomyelia
Foramen maganum compression syndrome
How does central chord syndrome present?
It is characterized by loss of motion and sensation in arms and hands - Upper limb affected more than lower limb
Which syndrome causes is damage to one half of the spinal cord, resulting in paralysis and loss of proprioception on the same (or ipsilateral) side as the injury or lesion, and loss of pain and temperature sensation on the opposite (or contralateral) side as the lesion.
Brown Sequard syndrome
Type II Chiari malformations are when parts of the cerebellar vermis and medulla and 4th ventricle protrude below the foramen magnum. It is almost always associated with ______
Myelomeningocoele
What are common clinical features in Type II Chiari Malformations?
Findings due to brainstem and lower cranial nerve dysfunction
Most common type/angle of craniosyntosis?
Sagittal!
Sagittal Craniosyntosis results in what shaped head?
Long narrow shaped head
What are common clinical features of Craniosyntosis?
Skull deformity, Raised ICP, hydrocephuls
May also get opthalmologic problems due raised ICP to bony abnormalities of the orbit.
When do you really need surgery for Craniosyntosis?
More than 2 sutures involved
What are key differentiating features between deformative plagiocephaly and craniosyntosis?
Deformative plagiocephaly results in normal growth rate of head, parallelogram shaped head, Nose generally straight, ears anterior and there will be external pressures
Craniosyntosis - rhomboid shaped head, nose pointing towards fused suture, ears posterior, slow growing head!
How do you manage Deformity plagiocephaly?
Managed by GP or paediatrician. Counter
positioning
and/or helmet
therapy
How do you manage Craniosyntosis?
Craniofacial surgeon - expansion surgery.
60% of all paediatric brain tumours are _______
Infratentorial
