Category Archives: Neurology

Stroke in children

Rare but happens.

Differential:

Can be due to arterial or venous occlusion.  50:50 in kids cf adults (80% infarct). Haemorrhagic can be due to rupture into infarct.

Presents with focal signs, headache, seizures most commonly. Else dysphasia, vomiting!, confusion. Fever! Acute signs often lacking or fluctuant cf history!  FAST criteria only 78% sensitive. 

NIH stroke severity scale has paeds version. 

Risk factors

Black/Asian

Cardiac (esp surgery, right to left shunt)

Sickle cell – esp anaemia, acute chest syndrome, HbS or HbS/Beta thal

Thrombophilia

Liver/kidney disease (secondary prothrombotic tendency)

VZV within 1yr, enteroviruses, HIV.

Vasculitis – Moya Moya (peaks at 5-9yr else adulthood), SLE, other

Cocaine, glue.

Marfans, homocysteinuria, Fabry’s disease, Neurofibromatosis

Cancer, radiotherapy

Hypoglycaemia. 

Management

High flow O2, 10ml/kg saline 

Imaging within 1hr. 

BP – avoid high and low? Cf adults

Monitor for RICP

Treat with aspirin.

Tests

  • CTA/MRA at time of CT/MRI
  • Echo
  • (Transcranial doppler in sickle cell- via temporal bony window)
  • Hbopathy screen
  • Cholesterol
  • Lupus anticoagulant, Anti cardiolipin ab (ACLA), consider beta 2GP1
  • Homocysteine
  • Alpha galactosidase
  • Lipoprotein A – marker for CVS disease, genetic. 
[RCPCH guideline May 2017]

Hypokalaemia

Could be reduced intake but usually excessive losses –

RenalNon-renal
Renal tubular acidosis (type 1 or 2)Vomiting eg pyloric stenosis
Bartters or Gitelmans syndromeDiarrhoea
DiureticsLaxative overuse
Hyperaldosteronism (CAH, tumour)Thyrotoxicosis
Salbutamol
Familial periodic paralysis
Pseudo-Bartter’s
Trauma
Diabetic ketoacidosis

Symptoms depend on severity and how rapidly decrease has happened. Chronic low levels are better tolerated. Since potassium important for membrane potentials, effects are mostly neuromuscular.

  • Cramps, weakness, paralysis
  • Ileus
  • Metabolic acidosis (although underlying cause often produces alkalosis)
  • Arrhythmia, heart failure
  • Rhabdomyolysis

ECG classically shows U waves, T wave flattening, and ST-segment changes. Can be tall wide P waves, can look like long QT if T and U waves merge.

Do urine and blood electrolytes to look at fractional excretion.

[Endocrine connections 2018][Current Treatment Options in Peds 2022]

Rett Syndrome

Exclusively females (lethal in males? Or rate of germ cell mutations higher in male germ cells?).  Virtually always sporadic – so not exactly X linked dominant.

MECP2 gene on X chromosome.

Developmental arrest at 6-18 months, then regression, loss of speech, stereotypies esp hands.

Often epilepsy, then complications of severe neurodisability eg chronic lung issues.

Not degenerative however – can live into middle life.

Focal epilepsy

As opposed to generalized epilepsy. Potentially associated with a brain lesion (congenital or acquired), but not always. EEG will usually confirm.

Associated with prolonged or focal febrile convulsion – chicken or egg?

See:

Temporal lobe epilepsy

The most common focal epilepsy.  Sense of déjà vu, phantom smells, panic attacks as possible aura symptoms! Talking gibberish, lip smacking, staring, posturing are more obvious.

Treatment

Levetiracetam or Lamotrigine first line, the former (Keppra) can be loaded more quickly though.

Honey in medicine

Contains a range of different sugars, aromatic oils, also pollens and bee proteins. Royal jelly and beeswax related, of course.

High fructose content can cause GI intolerance in some.

Allergic reactions can happen, often unrecognised, mostly due to specific pollens (depending on what flowers the bees feed on), in minority to bee proteins. Commercial honey tends to contain v low amounts of pollen, due to production techniques. IgE test for honey is available, but you may need to skin prick test with the specific honey if negative.

Honey eaten all year round is rumoured to prevent hay fever symptoms because of the pollens it contains, but this has not been proven, although it’s a nice idea related to immunotherapy. Depends on getting the right pollens of course – bees don’t like grass and birch flowers, probably. In some it may just trigger allergy symptoms.

Cross reaction between honey and bee venom is reported, not surprisingly, but not automatic.

Plant toxins can be present in sufficient quantities in honey to cause poisoning eg rhododendrons (some species).

Botulism reported in infants – failure to thrive, hypotonia, cranial nerve palsies. Clostridium and other bacteria cannot grow in honey due to the high sugar content, but spores can be present. So advice is not to give honey to infants.

Dravet syndrome

Previously Severe myoclonic epilepsy of infancy. Charlotte Dravet described in 70s. Characterised by:

  • Refractory epilepsy
  • Onset in infancy
  • Associated neurodevelopmental problems

Due to defect in SCN1A gene on chromosome 2q24 (a sodium channel), usually de novo. Many mutations, don’t predict severity, unfortunately.

Accounts for about 7% of epilepsy presenting in first 3 years of life.

Onset around 5-8 months, often with febrile illness so can look like typical febrile convulsion. But often prolonged. Neurodevelopmental problems come later…

Later though, multiple seizure types. Hypotonia, ataxia, spasticity all seen. Dysautomnia can be a feature. ADHD and autistic traits common later.

EEG can be normal, or vary over time, with multifocal or generalised changes, photosensitivity too.

Prolactin

Secreted from the anterior pituitary (along with growth hormone, ADH, ACTH, TSH, FSH/LH), but also a stress hormone (can be used to distinguish pseudo seizures from epileptic seizures). So can go up to 1000 in healthy people. Always worth repeating a high result at least 24hrs hours later, after a 20 minute rest.

Important because of prolactinomas, which can cause:

  • gynaecomastia
  • galactorrhoea
  • delayed puberty
  • space occupying lesion effects – headaches, visual field defects

Any lesion in the vicinity of the pituitary may also cause raised prolactin so not specific.

In children, high levels can be due to presence of macroprotein isoforms, which are not considered pathological – lab can check.

Arnold Chiari malformation

Thought to be congenital but often only picked up in adulthood when symptoms develop.

Type 1 most common, where cerebellar tonsils protrude into spinal canal, potentially putting pressure on brainstem, spinal cord and cause obstruction to flow of cerebrospinal fluid.

Can be found incidentally. Symptoms however include:

  • headache (especially occipital)
  • neck pain
  • numbness or paraesthesiae of fingers, arms, lower limbs
  • Coordination problems, dizziness

Can be complicated by upper spinal syringomyelia.

Rarely familial.