Draft guidelines for the management of alternating he miplegia
These guidelines outline the current position about diagnosis and management. They
should be read with the accompanying article written on treatment and management.
The diagnosis of AHC is usually delayed from the first evidence of the condition but
the combination of recurrent attacks which do not fit another diagnosis eg. epilepsy
and/or do not respond to treatment should prompt referral to a paediatric neurology
department with facilities for EEG, MRI, metabolic investigations and specialists in
child development. This referral from a general paediatric department may be
through a number of different arrangements (visiting clinics, direct referral) but it is
usual for the local community based team to remain part of the medical/therapy input.
On the basis of the data available on the 7 criteria, the paediatric neurologist may be
able to conclude that
the diagnosis is typical AHC
The investigations performed for episodic or fixed neurological impairments are
usually normal or non-specific in AHC.
It is now usual practice to perform video telemetry on attacks of uncertain nature in
young children even if AHC seems the likely diagnosis. This will often mean referral
to a specialist epilepsy unit.
Because of the rarity of the condition and lack of positive investigative findings it is
quite common to seek a second opinion either about diagnosis or management.
2. After diagnosis
Once the diagnosis of typical, atypical or possible AHC is made, the following should
The family should receive information about the condition both as a paper in their
language and the opportunity for discussion.
A programme of developmental surveillance should be initiated incorporating motor,
cognitive and behavioural dimensions. This should involve the use of simple multi-
domain questionnaires to the parents/carers.
Consideration should be given to preventative treatment and management of episodes.
This involves the use of drugs for prevention eg. flunarizine, avoidance of pro voking
events and early intervention with eg. sleep induction in events. There is sufficient
evidence of accumulated impairments following prolonged episodes to justify a
vigorous treatment schedule. It is usual, particularly early in the condition, for
children to be admitted during severe episodes giving parents and the medical team
the opportunity to share the experience and plan future management. Since severity
can vary and include times of respiratory compromise and inability to take fluids, it is
usual to offer automatic admission for this condition. This is discussed later in more
Information about support services should be given including social services and
voluntary parent support group.
Since 50% have true epileptic seizures, the semiology of the attacks must continue to be
documented since epileptic seizures may be a later complication. The management of
epileptic seizures is not specific for AHC and often not every effective.
Poor nutrition is very common in AHC. Paediatric review every 3-6 months should
include weighing and measuring and enquiring about feed ing. A drop of more than 1SD.
of weight against height should initiate an enquiry and intervention about nutritional
intake and more than 2SD. Involvement of dietician and consideration of supplementary
feeding. Because feeding is often impaired in attacks, the review of feeding should
assess the effect of attacks and safety of feeding in attacks which usually will have to be
done by observation rather than using a pre planned cine-swallow.
Children with AHC have relatively high rates o f cognitive impairment, ADHD, ASD,
combative behaviour, coordination disorder and problems with sleep. They are also more
prone to depression and anxiety than the normal population. Simple screening tests for
these problems exist and an enquiry about behaviour should occur once per year and
should include an account of a typical day, so that all issues concerned with sleep,
feeding, drug taking, bathing, dressing, etc can be reviewed.
Behavioural support/intervention should be available and coordinated with regular
paediatric care. This is easily written but often difficult to find. Some general points are
therefore worth making:
Cognitive assessment and appropriate educational provision are an essential part of the
management of problems in behaviour.
Behavioural history taking has to recognise the relationship to attacks of AHC.
We know of no reason why behavioural management should be different to other children
with significant early neurological problems. Specifically, we know of no contra-
indication to the use of drug treatment for ADHD, depression and anxiety if otherwise
judged to be appropriate.
Episodes of AHC
The initial episodes are likely to be misdiagnosed and usually cause hospital admission.
The separation of epileptic from AHC attacks is important and usually requires EEG
monitoring. Once the diagnosis is clear it is important that an agreed plan of
management is developed between parents and medical advisers. Most short attacks do
not require admission to hospital, but longer episodes, particularly bilateral ones may
need admission: this is particularly the case when the child’s ability to drink is impaired
although many parents learn to cope with this by using the ‘window’ of normal
functioning on waking. Problems of breathing in bilateral attacks necessitate admission
and may require assisted ventilation. It is important to identify breathing problems by
monitoring oxygen saturation during some bilateral attacks (see inpatient assessment).
Other indications for admission are concern about respiratory infection and severe
The usual acute treatment of severe episodes, ie. episodes lasting longer than 20-30
minutes and those which the family recognise early as likely to be more severe is the
rapid induction of sleep. Both oral melatonin, buccal midazolam and rectal diazepam are
used and a combination of melatonin and benzodiazepine may be used. It is suggested
that this should be tried initially as an inpatient to refine the dose and check for
hypoventilation. There is a balance to be struck between the amount of benzodiazepine
sleep induced and the likely severity/benefit of their use. Many parents are concerned
that children may lose abilities in severe attacks and particularly in younger children this
may appear to be the case and the need for acute treatment should be discussed.
Long term preventative measures: evidence for the efficacy of the drug prophylaxis is
not good and is summarised in the treatment paper. The commonly used drug is the
calcium channel blocker flunarizine which rarely stops attacks but is reported to reduce
the length and severity of attacks. The dose regime is from 5-20 mg/day and side effects
are very uncommon.
The other mainstay of prevention is to avoid precipitating factors: exposure to cold,
emotional stress, fatigue, bathing, high and low body temperature and upper respiratory
infection. Parents recognise if any of these are important and usually take sensible
precautions. However, these may be excessive and deprive the child of useful
experience. This matter needs to be discussed and reviewed.
It is important that the school is fully informed of the continuing problems, their
variability, attacks and their management. Cognitive assessment and screening for
common psychiatric disorders (see behaviour above). There must be clear individual
guidelines for the management of acute episodes and if encouragement of sleep is
required, the details of when, who and where this occurs need to be negotiated. The
school will need an available paediatrician as well as parents for such matters.
Educational progress is monitored as for any child by academic progress, assessment of
their participation and attendance in school and its activities.
There are a wide range of motor problems which require identification and ma nagement.
Mobility in general is managed in the normal fashion with the exception of needing to
provide for the child’s condition in attacks even if these only occupy a minority of the
Problems of dyspraxia, dystonia, chorea, tremor, weakness and spasticity may benefit
from occupational therapy input and occasionally drug treatment may be helpful in
dystonia, tremor and spasticity. Surgical treatment is rarely used.
Need for inpatient assessment
With a condition as rare and as disabling as AHC there is a strong case for a period of
assessment providing the aims are clear and achievable. These aims can include:
The nature of the attacks (AHC or epilepsy)
Oxygen saturation in severe episodes
The best method of achieving sleep
Exploring problematic behaviour
Such assessments may require a combination of a planned and acute admission.
Long term s upport
There is no doubt that this condition with its variability, episodes and multiple
manifestations puts great stress on parents and other carers. The elements of good
Well thought out individual guidelines reviewed annually
A local paediatrician/disability team that take full responsibility
An available specialist unit for specific problems
Fully integrated educational, social and medical services
Within the social services there should be awareness of the team of the indications
for a social services assessment and the availability of appropriate in- home
support and respite care.
Brian Neville August 2009
C:Neurosciences Documents\Brian Neville\Guidelines for the management of alternating hemiplegia