Neurology Flashcards
What should be done in a consultation with a child with headache?
Growth parameters, OFC, BP Sinuses, teeth, visual acuity Fundoscopy Visual fields (craniopharyngioma) Cranial bruit Focal neurological signs Cognitive and emotional status
What clinical symptoms indicate a childhood migraine?
Associated abdominal pain, nausea, vomiting
Focal symptoms/ signs before, during, after attack: Visual disturbance, paresthesia, weakness
‘Pallor’
Aggravated by bright light/ noise
Relation to fatigue/ stress
Helped by sleep/ rest/ dark, quiet room
Family history often positive
What are headache signs of raised ICP?
Aggravated by activities that raise ICP eg. Coughing, straining at stool, bending
Woken from sleep with headache +/- vomiting
What are the indications to do neuroimaging?
Features of cerebellar dysfunction Features of raised intracranial pressure New focal neurological deficit eg. new squint Seizures, esp focal Personality change Unexplained deterioration of school work
What drug can be used for prophylaxis of tension type headache in children?
Amitriptyline
What are some non epilepsy induced seizure types?
Acute symptomatic seizures: due to acute insults eg. Hypoxia-ischaemia, hypoglycemia, infection, trauma
Reflex anoxic seizure: common in toddlers
Syncope
Parasomnias eg. night terrors
Behavioural stereotypies
Psychogenic non-epileptic seizures (PNES)
What drugs can be used for childhood epilepsy?
Sodium Valproate (not in girls) or Levetiracetam: first line for generalised epilepsies
Carbamazepine: first line for focal epilepsies
Several new AEDs with more tolerability and fewer side effects: Levatiracetam, Lamotrigine, Perampanel
Other therapies: steroids, immunoglobulins and ketogenic diet (mostly for drug-resistant epilepsies)
What is microcephaly?
Definition: OFC <2 SD: mild
OFC <3 SD: moderate/ severe
Microcephaly usually indicates small brain: ‘micranencephaly’
What is macrocephaly?
Definition: OFC > 2SD
Look for causes in brain, sutures and fontanelles
What are some abnormal head shapes in children?
Plagiocephaly ‘flat-head’
Brachycephaly ‘short head or flat at back’
Scaphocephaly ‘boat shaped skull’
Craniosynostosis-baby’s skull joins too early so is misshapen
What signs will a baby show if they have a possible neuromuscular disorder?
Baby ‘floppy’ from birth Slips from hands Paucity of limb movements Alert, but less motor activity Delayed motor milestones Able to walk but frequent falls
What kind of inheritance does Duchenne’s muscular dystrophy have?
X linked inheritance
What is the presentation of Duchenne’s?
Delayed motor milestones Calf hypertrophy Ambulation difficulty and falls Diminished muscle tone and deep tendon reflexes Normal sensation Gowers' sign-The relatively weaker hip extensors, knee extensors, and ankle dorsiflexors result in the patient 'climbing up his body' to come to stand from a seated position. Toe walking Waddling gait Hypotonia Hyperactivity Urinary and bowel incontinence Mild to severe mental retardation
What will be seen on investigations in Duchenne’s?
Serum CK-raised
Genetic Testing-Xp21 mutation can be seen
Muscle biopsy-absent dystrophin
Electromyogram
What is the difference between Becker and Duchenne dystrophy?
In Duchenne dystrophy, this mutation results in the severe absence (< 5%) of dystrophin, a protein in the muscle cell membrane. In Becker dystrophy, the mutation results in production of abnormal dystrophin or insufficient dystrophin.
Becker’s muscular dystrophy is less severe than Duchenne and weakness only becomes apparent in young adults.