Category Archives: Neurology

Living with Epilepsy

General paediatrics, Neurology

Driving

In UK, driving is forbidden after a single epileptic seizure.  If seizure happens after age of 5, permanent ban on driving HGV, passenger service vehicles, racing.

Reinstatement of license depends on seizure free period, and whether seizures have occurred only during sleep or not.  You can reapply if you haven’t had an attack for at least a year.  If you had a seizure because your doctor changed or reduced your anti-epilepsy medicine, you can reapply when:

  • the seizure was more than 6 months ago
  • you’ve been back on your previous medication for 6 months
  • you haven’t had another seizure in that time

If your seizures have only been during sleep, you need at least 12 months seizure free.  If seizures have been when awake as well as in sleep, then must have been 3 years with only seizures in sleep.

If however generalized spike and wave activity on EEG, then reinstatement cannot occur.  DVLA seem to retain the right to decide on individual basis.

Death and Accidents

SUDEP (sudden unexpected death in epilepsy) should be discussed at or around the time of diagnosis.  There is a tendency for professionals to avoid talking about it.  Evidence from review of cases is that deteriorating seizure control in preceding 3-6 months common, as is lack of regular clinic review.

Discuss safety esp bathing, swimming, cycling.

There are numerous devices and tools available.  Unfortunately, no single type of monitor (movement, breathing, ECG, EEG) likely to be sensitive.  But important to have balanced approach – consider risk of events, likelihood of false alarms, and the fact that no device can guarantee that SUDEP will not occur.

The best way to prevent SUDEP is to stop seizures occurring in the first place!

BMJ safety checklist

EpSMon is a patient app.

Another useful source of objective information is the latest Cochrane Review on SUDEP interventions.

Other things to consider:

  • Medical ID cards or Jewellery
  • Seizure alert dogs
  • Seizure alarms – phone app, else local authority may be able to provide, else charities
  • Anti-suffocation pillows
  • protective headgear

In a UK review of epilepsy deaths.  15% related to status epilepticus, 15% SUDEP.  Majority due to co-existing morbidities.  24% considered preventable, usually related to fragmentation of care, support for families in responding to emergencies, and hospital response to acutely unwell child (incl status). [BPS poster]

Contraception

Women taking anticonvulsants and oral contraceptives have 25 times the risk of pill failure as normally expected, due to action of some anticonvulsants (eg phenytoin, phenobarbitone, primidone, carbamazepine, and ethosuximide) on the hepatic microsomal enzyme system, resulting in a dramatic decrease of circulating oestrogens.  Discuss with pharmacist, as complicated!

Contraception also important if potential teratogenic effect esp valproate.

Family Support

Autistic Spectrum Disorder

Community, General paediatrics, Mental health, Neurology

ICD-10 defines autism spectrum disorder as

  • persistent difficulties with social communication and social interaction, and
  • restricted and repetitive patterns of behaviours, activities or interests (this includes sensory behaviour),
  • present since early childhood,
  • to the extent that these limit and impair everyday functioning

Sensory behaviour may be meltdown or withdrawal or other challenging behaviour when too much information or sensation is experienced.  There can be hyper (or hypo) sensitivity to lighting, problems with depth perception, noises or crowds, smells (or licking), pain, taste/textures.

SIGN guidance is that (145):

  • children under 3 with regression in language or social skills should be referred
  • not to screen population
  • that screening instruments are not 100% reliable but have their uses
  • that ASD should be considered in any child with developmental, emotional, psychiatric or behaviour issues, or a genetic syndrome
  • in preschool children typical features may be absent
  • gender differences are important in terms of symptoms and level of impairment

Assessment

  • Lack of shared attention (or late development) eg pointing
  • repetitive behaviour/play
  • resistance to change
  • violent or self injurious behaviour, pica

Types

  • Aspergers – social difficulties in absence of learning or communication problems
  • Pathological demand avoidance – where underlying problem is high level of anxiety about conforming to social demands or not being in control

Support

ARCH, REACH and National Autistic Society

Stereotypy

Clinical, General paediatrics, Neurology, OPD

Where children present with abnormal movements, consider:

Stereotypies are repetitive non-functional movements, typically hand flapping or twisting, body rocking, head banging/nodding, grimacing, arm flapping. As with tics, there is often a family history, and there is an association with obsessive compulsive tendencies.

They can be present in children with normal development, but are a feature of neurological disorders especially autism spectrum disorder and sensory impairment.  In these children, the movements are part of a period of introspective absorption, they make prefer such activity to conventional social interactions.

There are a number of differences from tics, although they can co-exist:

  • Sterotypy presents younger, eg under 2 yrs.  Tics present from 4 onwards.
  • Tics can vary over time, so grimacing moves on to  shoulder jerks, then moves on to clearing throat.  Stereotypy movements are unchanging.
  • Stereotypy movements are rhythmic, rather than just a single jerk
  • Tics are brief, stereotypy can be prolonged
  • Tics have a premonitory sensation (although only older children may be aware)
  • Tics can be suppressed with effort.  Children with stereotypy can be distracted but may resent it! (Similarly self gratification)

Excitement and stress are triggers for both.  Over time, the child usually becomes aware of social disapproval and may suppress the behaviour except in secret!

[Ulster Med J 2014;83(1):22-30]

 

 

 

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, girls more than boys
  • Abrupt onset and cessation
  • Stare into space, unresponsive for around 10 seconds
  • Afterwards just carry on unaware anything has happened – or possibly very brief confusion (seconds)
  • Clue that not just day dreaming is that most also have 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.
  • Usually when sat quietly rather than actively engaged in something. Precipitated by sleep deprivation, as other epilepsies, but also hyperventilation (90% detected after 3 minutes – get them to count aloud)
  • Atypical would be gradual onset, last longer, have more obvious changes in tone – but continuum
  • Most genetic. GLUT1 (glucose transporter protein type 1, coded for by SLC2A1 gene, involved in glucose transfer across blood-brain barrier) mutations are present in up to 10% of early childhood absence epilepsy (ie under 5yrs). Heterozygous mutations are mostly de novo but may be inherited as AD trait. SLC2A1 positive often not helped by valproate and ethosuximide, whereas ketogenic diet often 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