Category Archives: Neurology

Cluster headache

General paediatrics, Neurology

Very rare – unilateral, orbital, supraorbital and/or temporal. Pain lasts 15-180mins but attacks occur multiple times a day (or at least on alternate days). Associated with autonomic features eg ipsilateral conjunctival injection, nasal congestion, forehead sweating, miosis, ptosis, agitation. Peak incidence in 20s, males predominate.

Can be episodic (bouts lasting 6-12 weeks every 1-2years) or else chronic.

Treat with high flow O2! Sumatriptan SC better than nasal, Zolmitriptan nasal preferred to oral or sumatriptan. Safe, licensed for kids.

Prophylaxis in adults is with verapamil!

Tension headache

Common, General paediatrics, Neurology, OPD

Tension headache

  • Mild to moderate rather than severe,
  • pressing or tightening rather than pulsatile,
  • Bilateral,
  • Not aggravated by routine physical activity.

Can be continuous. Phonophobia, photophobia, nausea are possible, but if more than one present, and particularly if vomiting or severe nausea, then migraine would be preferred diagnosis.

Often spreads into or arises from neck.

Chronic tension-type headache – as above but on >/15 days/month for at least 3 months. But gets messy – it is possible that a patient can have both this and Chronic migraine, viz only two of the four pain characteristics are present and associated with mild nausea. In all these cases consider Medication-overuse headache.

Idiopathic intracranial hypertension

General paediatrics, Neurology, Surgical

Previously “Benign” Intracranial Hypertension but not entirely benign…

Intracranial hypertension but with normal CSF, and no ventriculomegaly. Presents with usual symptoms of early morning headache, effortless vomiting. VI nerve palsy may be seen, rarely II/IV. Papilloedema is often the first clue.

No sex differential prepubertally, not associated with obesity (contrary to popular belief).

Normal CSF opening pressure is 7.5cm of water <2yr, 13.5 <5yr, 20 over 5yr. Lumbar puncture is therapeutic; 2 step tap procedure is usually used if opening pressure is over 30cm. NB General anaesthetic can give false pos result! Secondary causes include drugs, endocrine conditions.

Since repeated LP is unpleasant, medical therapy can be considered. Topiramate is probably equivalent to the more usual acetazolamide (a diuretic). Steroids should be used for malignant hypertension (ie where there is rapid progression). Any of these treatments may result in a low pressure headache.

Surgical options include Optic nerve sheath fenestration, lumbar-peritoneal shunt.

Headache

Common, General paediatrics, Neurology, OPD

Common problem in children, as well as adults!

Distinguish primary from secondary.

Most headaches get worse with exertion so that’s not a discriminating feature.  Headaches that get worse on standing suggest a CSF leak; worse on lying down suggests a tumour.  See NICE CKS.

Primary

Secondary

Migraine

Common, General paediatrics, Neurology, OPD

International Headache Society 2004 Migraine without aura def:

  • A – at least 5 attacks fulfilling B-D
  • B – lasting 1-72hr
  • C – at least 2 of:
    • unilateral, may be bilateral frontotemporal but not occipital;
    • pulsing;
    • moderate or worse pain;
    • aggravation by routine physical activity eg walking, stairs
  • D – during headache at least 1 of: nausea +/or vomiting, photophobia and phonophobia (which may be inferred from behaviour)
  • E – not attributed to other disorder

Aura – Hemianopia or spreading scintillating scotoma. Note that migraine with aura is a contraindication to treatment with combined oral contraceptives.

Some specific types:

  • Hemiplegic – can be familial or sporadic.  Can be confusion.  Rare to not have headache with it (but then diagnosis perhaps not recognised!?).  Triptans were initially thought to be risky, but more recently good evidence of usefulness and no longer contraindicated in BNFc.
  • Ocular/retinal – blindness or flashing lights, may not be headache.  NOT aura, which is prodromal.  Horner’s syndrome seen.
  • Basilar (also called Bickerstaff’s – but better termed migraine with brainstem aura) – transient dysarthria, vertigo, tinnitus, hearing impairment, diplopia, ataxia, confusion, bilateral paresthesia,
  • Confusional

Pathology

Neuronovascular condition – baseline hyperexcitability in cortex.

Double the risk if you had infant colic, which is not true for tension headache!  Sleep disruption as common factor? [JAMA 2013;309:1607-12]

Several genetic links found eg C677T mutation of the methylenetetrahydrofolate reductase gene (MTHFR), EAAT2. [J Headache Pain. Jan 2012; 13(1): 1–9.  doi:  10.1007/s10194-011-0399-0]

Investigations

Beware Occipital epilepsy!

American Academy of Neurology recommendations are that neuroimaging should be considered in:

  • recent onset of severe headache;
  • change in type of headache;
  • or neurological dysfunction;
  • seizures

Factors

  • Biofeedback and relaxation/stress management are as effective as beta blockers. Indian trial of yoga for migraine showed substantial improvement at 3 months [neurology 2020]
  • Sleep disturbance is associated but not necessarily causal – so recommend good sleep hygiene.
  • Exercise is beneficial.
  • Obesity – clusters with diet, exercise, sleep issues of course.
  • Missing breakfast is a common precipitant.
  • Episodic migraine (without aura) can become chronic (ie 15 days per month or more), but in this case you would always want to exclude medication overuse.
  • Caffeine is linked to headache and also sleep/mood disorder which exacerbates. Withdrawal headache can last as long as a week.
  • Wine is well recognised trigger in adults!
  • Often linked to menstrual cycle.
  • Screen time (>2hrs per day) linked to migraine but not non-migraine headache in French and Sri Lankan students.  Doesn’t necessarily mean reducing screen time helps, of course.  But note eye strain, posture relevant too.  And possibly differences between TV/PC use and mobile devices.
  • Some evidence for magnesium and zinc supplementation

[CurrOpPeds Dec 2004]

Diet

Although popular perception is that migraine is caused or at least triggered by dietary factors, there is a wide variation in reporting of dietary triggers. Certainly migraine is associated with obesity, and dietary habits seem to be as important as specific foods, and there is probably a cycle of inconsistent nutrition and poor control of migraine.

My colleagues talk about 4Cs (chocolate, cheese, coffee, citrus) but I have found no evidence for this.  Awareness of possibility of dietary triggers actually has greater influence on perception of personal triggers than personal experience!  See below.

Some evidence for lipid intake esp PUFA (decreased ingestion of lipids was associated with a decrease in the frequency, intensity, and duration of migraines and a decrease in the use of medication) but confounded by obesity, weight reduction, and changes in nutrient intake.  Another study found reduced migraine spells among children subjected to a diet rich in fibre. Not much evidence for cheese at all! [Nutrition Reviews. 70(6):337-56, 2012 Jun.  UI: 22646127]

Trial of cyanocobalamin, folate, and pyridoxine (2 mg of folic acid, 25 mg vitamin B6, and 400 microg of vitamin B12) in patients with MTHFR gene defects found a reduction of homocysteine levels and improvement of migraines. [Pharmacogenetics and Genomics. 22(10):741-749, October 2012]

Medication

Paracetamol, ibuprofen, and nasal-spray sumatriptan are all effective symptomatic treatments for episodes of migraine (peds sys rv 2005). Migraleve is paracetamol, codeine (8mg), buclizine – for 10yr plus. Paramax, with metoclopramide, for 12 yr plus.  Anti-emetic improves pain killer absorption so potential benefit even if no nausea!

Sumatriptan was previously contraindicated for hemiplegic migraine, but this was probably a theoretical concern, and there is evidence that it works. No caution or contraindication mentioned in BNF now.

Mefenamic acid for menstrual, esp with dysmenorrhea.

CGRP receptor antagonists – olcegepant, telcagepant [not in BNF].  Now erenumab [monthly subcut injections, BNF says specialist use only, minimum 4 migraines per month – SMC approved with restrictions], eptinezumab etc vs same calcitonin gene-related peptide receptor.  Significant improvement in headaches in 40%, about 3 less headache days per month.

For prophylaxis, Pizotifen does not work (grade I evidence, plus makes you fat), flunarizine (CCB) is the most effective (not in BNF), also amitriptyline (dangerous in overdose), propanolol (dizziness, sleep disturbance, depression etc, also dangerous in overdose). Encouraging data (grade IV) for anti-epileptic mediations topiramate, valproate, levetiracetam, zonisamide. Cyproheptadine? Candesartan (anti-hypertensive, in BNF for migraine prevention)?

Always increase preventer dose to maximum before giving up, note that often symptoms worsen after initial benefit.

Current evidence on the efficacy of percutaneous closure of patent foramen ovale (PFO) for recurrent migraine is inadequate in quality and quantity. The evidence on safety shows a small incidence of well-recognised but sometimes serious adverse events, including device embolisation and device prolapse (each reported in less than 1% of patients). Therefore this procedure should only be used with special arrangements for clinical governance, consent and audit or research. [NICE]

Contraception

Combined oral contraceptives are contraindicated in migraine with aura.  But may be useful if menstrual pattern to headaches if no aura.

Prognosis

50% migraine in childhood remits at puberty. Onset in adolescence associated with persistence.

Support

Patient support at Migraine Trust.

Childhood absence epilepsy

General paediatrics, Neurology
  • Age 3 to 10 yr
  • Abrupt onset and cessation
  • Most are complex ie clonic movements, minor changes in tone (eg head drops, or held object dropped),
    automatisms (repetitive movements of eyes, mouth).
  • No myoclonus (else likely to be juvenile myoclonic epilepsy). Some get generalised tonic-clonic seizures in adolescence but these are infrequent and respond well to treatment.
  • Precipitated by flashing lights, sleep deprivation, hyperventilation (90% detected after 3 minutes – get them to count aloud)
  • Atypical have more gradual onset, last longer, have more obvious changes in tone – but continuum
  • The SLC2A1 gene codes for the glucose transporter protein type 1 (GLUT1), involved in glucose transfer across blood-brain barrier. Heterozygous mutations are mostly de novo but may be inherited as AD trait. There may be mild learning and motor delay, but more often it presents with early absence seizures – GLUT1 mutations are present in up to 10% of early childhood absence epilepsy (ie under 5yrs). The importance of the diagnosis is that the seizure are often intractable to valproate and ethosuximide, whereas a ketogenic diet will be effective.
  • EEG shows sudden onset 3Hz spike and wave, esp with photic stimulation/hyperventilation. Interictal is often normal.
    Clinically apparent if more than 3 seconds of activity, but detailed neuropsych assessment suggests that non-clinical absences do cause functional impairment. Atypical have slower spike waves and rarely have normal interictal.
  • 60-80% full remission, usually during puberty; in most cases, absences disappear on monotherapy but there are resistant cases (unpredictable, other than SLC2A mutations).

Occipital lobe epilepsy

General paediatrics, Neurology

Early (PANAYIOTOPOULOS) or BECOP (Benign epilepsy of childhood with occipital paroxysms).

Panayiotopoulos

  • Or Self limiting epilepsy with autonomic symptoms (SeLEAS)
  • Usually 3-5yrs but can be 1 to 15
  • Mostly nocturnal
  • Characteristically autonomic – pallor/flushing/vomiting/coughing!
  • Head/eye deviation, then tonic-clonic (one sided or generalized)
  • Often status, but actually seizures infrequent
  • EEG can be normal – sleep EEG more sensitive
  • Good prognosis – most remit with a few years and have just a few seizures
  • Valproate or carbamazepine

BECOP

  • Usually middle childhood, cf above
  • Can be triggered by going from dark to light areas or vice versa
  • Visual  – partial or complete loss, flashing lights, multi-coloured circles, balls, rarely hallucinations
  • Headache during or after – cf migraine!
  • Often one side jerks; rarely generalized
  • Carbamazepine or Valproate
  • Usually benign, most remit by puberty

Sacral dimple

Clinical, Congenital, General paediatrics, Neurology

Typical sacral dimples are <5mm in diameter, within 25mm of anus and located in midline.  Rate of spinal  dysraphism (bifida occulta) less than 1%.

Higher risk if do not fulfill these criteria. Lipomas, deviated/bifurcated crease are the most likely to be associated with dysraphism.  Otherwise you expect at least 2 or more cutaneous markers (hair tuft, haemangioma, Mongolian spot, skin tag/tail).

Reports of high frequency of hair tufts in diastematomyelia probably refer to more striking lesions (“faun tails”).

Royal College of Radiology has policy  – ignore sacral dimples unless atypical, or in combination with other lesions.

USS if neonate, but MRI if US abnormal or equivocal, where neurological signs (bladder, bowel, lower limb) or lesion discharging.

[Arch Derm 2004]

Episodic autonomic symptoms

Clinical, General paediatrics, Neurology, OPD

Dyspraxia, or Developmental Coordination Disorder (DCD)

Community, General paediatrics, Neurology, OPD

Developmental Co- ordination Disorder (DCD), as outlined in DSM IV (American Psychiatric Association 1994):

  • Performance in daily living activities that required motor co-ordination is substantially below that expected given the person’s chronological age and measured intelligence. This may be manifested in delays in achieving motor milestones (i.e. walking, crawling, sitting) dropping things, ‘clumsiness’.  Significantly interferes with academic achievement or activities of daily living.
  • The disturbance is not due to a general medical condition (e.g. Cerebral Palsy, Hemiplegia or Muscular Dystrophy), and does not meet the criteria for a pervasive Developmental disorder.
  • If Global Learning Difficulties are present the motor difficulties are in excess of those associated with it.

It is essential that early referral is made in order that children do not develop behavioural difficulties due to their frustration at not being able to carry out the same tasks as their peers.

Clues are:

  • Does the child’s motor skill appear to be behind their cognitive skills?
  • Dose the child appear to move generally in an uncoordinated way i.e. walking, running, manoeuvring around objects?
  • Does the child fall over constantly, bump into things, and /or knock thing over?
  • Has the child developed a dominant hand i.e. does he/she prefer to use one hand for more tasks?
  • Does the child have difficulties with dressing especially organising themselves? Do they find laces, small fastening and cutlery difficult?
  • What is their attention span like? Are they always fidgeting or squirming?
  • Are they having significant difficulties in the classroom in relation to their peer e.g. poor behaviour, avoidance of tasks, poor handwriting, dislikes gym?

N.B  Most children in their early school years will demonstrate one (or more) of these areas of difficulty but this does not mean they all have DCD!  Children with DCD will present with many of the difficulties above for a prolonged period.