Suspected neurological conditions: recognition and referral

Attention, concentration and memory problems related to epilepsy

The committee noted that concentration and memory problems are common in children who have diagnosed or undiagnosed epilepsy. They agreed that, in a child not diagnosed with epilepsy, episodes of loss of awareness, attention or concentration might be unrecognised absence seizures that need further investigation.

In children already diagnosed with epilepsy, the committee discussed drowsiness caused by anti-epileptic medicines, especially in higher doses or if more than 1 medicine is being taken. Children and their parents or carers can be advised about this, and it might be possible to adjust doses to reduce the effect on concentration and memory.

Concentration or memory difficulties that interfere with learning or behaviour

The committee agreed that concentration and memory difficulties that interfere with a child's learning should have further assessment to avoid unscheduled healthcare visits for learning difficulties in the future.

The committee discussed the common perception of children with attention and concentration problems as having hyperactive, noisy and destructive behaviour. Such children readily come to the attention of primary care, school and paediatric neurodevelopmental services. The committee wanted to ensure that children who do not have hyperactivity but do have significant attention and concentration problems are also recognised. Because they do not behave in a disruptive manner, these children may not be identified promptly, and may later present with learning difficulties.

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Blackouts and vacant spells

Blackouts in children can be caused by neurological disorders, predominantly epilepsy, cardiac disorders or simple syncope. The committee observed that, even with a clear first-hand description of the event, it is not always possible to make a confident diagnosis without specialist assessment and investigations. The committee agreed that the recommendation for people with suspected epilepsy in the NICE guideline on transient loss of consciousness ('blackouts') in over 16s is applicable to children aged under 16.

Vacant spells – often called absences – as a result of epilepsy in children can be difficult to distinguish from day-dreaming and loss of concentration, and need further assessment.

Blackouts in children under 12 years

The committee agreed that all children under 12 with blackouts or transient loss of consciousness should be referred for urgent assessment because history and examination do not always allow a diagnosis to be made confidently and there are a number of potentially serious causes that need to be excluded.

Vasovagal syncope

Vasovagal syncope is common in young people, and is often inappropriately referred because of concern that it represents seizures. The committee considered that the recommendation on vasovagal syncope in the NICE guideline on transient loss of consciousness ('blackouts') in over 16s is applicable to children aged 12 to 15 years.

Blackouts, seizures or amnesia after a head injury

Transient loss of consciousness after a head injury in children is usually immediate or within a few minutes. Much-delayed paroxysmal events after head injury in children – days or weeks later – are rare but warrant urgent referral for neurological assessment. The committee considered that the recommendations on pre-hospital assessment, advice and referral to hospital in the NICE guideline on head injury should be followed.

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Dizziness with no accompanying symptoms or signs

The committee wanted to reassure non-specialists that dizziness without any other symptoms is very unlikely to indicate a brain tumour. Brain tumours usually present with symptoms such as headache, nausea and vomiting, ataxia or drowsiness.

The committee also thought non-specialists should consider the possibility of migraine as a cause of dizziness. They agreed that clinical judgement should be used to determine the care pathway for children with dizziness and migraine.

Dizziness in older children

The committee thought that non-specialists should bear in mind the possibility of postural hypotension, which is common in children aged over 8 years. They noted that postural hypotension might not be present at the time of examination and cannot always be excluded by measuring blood pressure.

Dizziness caused by middle ear infection or effusion

The committee agreed that middle ear infection and middle ear effusion can be a cause of dizziness in children. They noted that the child may have fever, pain and diminished hearing, or a recent history of these, and that the eardrum might appear red and inflamed or bulging.

Recurrent dizziness

The committee identified recurrent dizziness in children as a red flag that warrants investigation once postural hypotension has been excluded. Cardiac dysrhythmias, although rare, can be a cause of dizziness, may be associated with exercise, and are potentially serious.

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Headaches in children under 12 years

The committee considered that children under 12 with headache and any of the 'red flag' symptoms detailed in this recommendation need immediate assessment, because these symptoms could indicate significant intracranial pathology, including a brain tumour.

Headaches in children under 4 years

The committee agreed that headache in a child aged under 4 years is an unusual symptom and, when present, has a high chance of being associated with a significant intracranial disease. Because the child is unable to articulate clearly what is wrong, parents may report excessive crying, a high-pitched cry or excessive irritability.

Recurrent headaches and migraines

The committee agreed that examination of the retinal fundus to identify disc swelling is essential for children with recurrent headache. They acknowledged that not all non-specialists have the skills needed for retinal fundus examination so they recommended that this could be requested, for example, from an ophthalmologist or optician.

The committee discussed raised blood pressure as a rare cause of headaches in children. The headaches may be accompanied by dizziness, vomiting or blurred vision. Because normal blood pressure changes with age and body height, they recommended that measurements of blood pressure in children are compared with standardised blood pressure charts adjusted for age and height.

Headaches that are relieved by lying down might indicate spontaneous intracranial hypotension. Although this condition is rare, the committee agreed that children with this symptom would benefit from referral and treatment.

The committee accepted that migraine is common in children and does not usually need referral. They agreed that stress can trigger migraines or chronic headaches. They also noted that overuse of analgesics can cause recurrent headaches.

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Children with dysmorphic features and developmental delay

The committee discussed a number of rare syndromes that involve premature closure of cranial sutures in association with other dysmorphic features, including disorders of facial growth and limb deformities. The investigation and management of these disorders is highly specialised and complex. The committee recommended urgent referral because early surgical intervention can be beneficial for these children.

Children aged under 4 years

The committee noted that abnormalities of head shape or size are most likely to indicate disorders of brain growth or raised intracranial pressure in children aged under 4 years, so measurement of head circumference is important in this age group if abnormalities are suspected. They agreed that GPs and health visitors should adopt standardised methods to measure head circumference and chart head growth, to ensure that accurate and consistent measurements are available to guide referral decisions.

Babies aged under 1 year with plagiocephaly

Babies frequently have a preferred lying position with their head to 1 side. This can lead to positional plagiocephaly, a benign condition in which 1 side of the head is flattened. The committee discussed how measuring the distance between the tragus of the ear and the outer canthus of the eye is a useful adjunct to clinical inspection and can help to reassure parents or carers. However, the committee acknowledged that this is not an absolute discriminator. Clinical assessment is always subjective and there is no test, short of imaging (rarely justified in plagiocephaly), that is infallible. Therefore, if there is uncertainty, referral for specialist assessment is advisable.

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Sudden-onset or progressive limb or facial weakness

In the committee's view, sudden or rapidly progressive limb or facial weakness in a child is usually a symptom of a pathology that needs immediate neurological investigation or management because the child's condition can deteriorate rapidly.

Limb weakness as part of a developmental disorder

The committee noted that cerebral palsy is the most common chronic motor disorder that affects children, but other genetic and medical disorders can have a similar presentation. They agreed that the recommendations in the NICE guideline on cerebral palsy in under 25s should be followed.

Boys with limb weakness

See the rationale on early diagnosis of Duchenne muscular dystrophy.

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New-onset gait abnormality

The committee agreed that new-onset gait abnormality could indicate trauma, infection, appendicitis or a hip abnormality, so these children need to be referred immediately.

Motor development delay

Although most children with motor development delay are simply at the slower end of the normal range of acquisition of motor skills, a small number will have a neurodevelopmental disorder such as muscular dystrophy or cerebral palsy. Assessment is relatively quick and the committee's view was that it is worth referring these children to a child development service to screen for problems and, in most cases, reassure parents. The committee agreed that the NICE guideline on cerebral palsy in under 25s provides further advice on referral for these children.

Early diagnosis of Duchenne muscular dystrophy is especially important so that the family can be offered genetic counselling. Creatinine kinase measurement is an inexpensive, routine test that can help identify this condition. If the test result is negative, Duchenne muscular dystrophy is unlikely.

Motor development regression

The committee agreed that specialist investigation of motor development regression is best carried out by a paediatric neurodevelopmental service or paediatric neurology because of the complexity of the investigations needed.

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Children with a recent head or neck trauma

Abnormal neck posture in a child who has had a recent head or neck trauma can indicate instability of cervical spine through bony or ligamentous injury, so the committee agreed that the child should be referred to an emergency department immediately, and immobilisation applied in line with the recommendations in the NICE guidelines on head injury and spinal injury.

Children with no recent trauma

The committee noted that an abnormal neck posture might be the result of painful enlarged lymph nodes that are a common presentation and can be managed in primary care.

The committee agreed that the most common cause of abnormal limb posture in children is pain or injury. If there is no history of an associated acute event or obvious musculoskeletal cause, the child needs to be referred to exclude progressive causes that need treatment.

The committee noted that abnormal head tilt can present before other typical symptoms of posterior fossa tumours, such as ataxia, vomiting and headaches. This finding is sometimes dismissed, leading to delay in diagnosis, and the committee therefore considered it useful to draw attention to the possibility.

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Tingling together with other symptoms

Tingling together with other peripheral nervous system symptoms might indicate pathology affecting the spinal cord. It is not easy to recognise this pathology on clinical examination, so the committee agreed that children with these symptoms need to be referred urgently.

Tingling can also be the first symptom of Guillain−Barré syndrome which, although relatively rare, can affect respiratory function through motor impairment, so the committee agreed that children with tingling and features suggesting motor impairment need to be referred urgently.

Isolated tingling, altered sensation or paraesthesia

The committee noted that transient, fixed-pattern sensory symptoms that are not associated with compression of a nerve can indicate epilepsy and that children with these symptoms should be referred. The NICE guideline on epilepsies provides advice on diagnosis and investigations for epilepsy.

Temporary tingling caused by nerve compression or hyperventilation

The committee agreed that temporary tingling of this nature is commonly caused by carrying heavy objects or over-breathing, and these children do not need to be referred.

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Symptoms suggesting possible respiratory failure

The committee noted that in children with neuromuscular disorders such as Duchenne muscular dystrophy, headaches on awakening in the morning can be an early sign of respiratory failure caused by hypoventilation during sleep. These children are also more susceptible to sleep apnoea and other breathing disorders caused by decreased muscle tone in the muscles of the airway.

Sleep disorders suggesting nocturnal seizures

The committee agreed that nocturnal seizures are a risk factor for sudden unexpected death in epilepsy (SUDEP) and need prompt investigation. They acknowledged that it can be difficult to identify nocturnal epileptic seizures, but highlighted that red flags are a stereotyped pattern of behaviour during the episode, an episode that starts as a focal seizure and then becomes generalised, or difficulty rousing the child after the episode.

Narcolepsy

The committee agreed that narcolepsy is a condition that is easily missed in children. It can present as daytime drowsiness, falling asleep in unusual circumstances, or as poor school performance and poor concentration. Diagnosis, assessment and management are best achieved by a service with experience of this condition, so the committee recommended referral to neurological services.

Sleep disorders suggesting sleep apnoea

The committee observed that sleep apnoea is not uncommon in babies and young children and might be caused by gastro-oesophageal reflux or intercurrent infection. In older children, it can be a result of enlarged tonsils and adenoids. Obesity can also cause sleep apnoea in children. They concluded that children with symptoms of sleep apnoea should be referred to determine the cause, and advice on weight loss should be offered for those who are obese.

Night terrors in children aged over 5 years

The committee noted that new-onset night terrors are unusual in children aged over 5 years, and could indicate epilepsy. For this reason, they agreed that new-onset night terrors in children of this age, as well as children in whom night terrors continue after age 12, should be referred.

Night terrors and other sleep disturbances in children aged under 5 years

The committee recognised that clinicians are aware that sleep disturbance is common in childhood and often resolves as the child gets older. However, reassuring parents that sleep disturbances are a normal part of development might help to reduce the pressure for an unnecessary referral. Referring children aged under 5 years to a health visitor may be helpful.

Sleep disturbances in children with neurodevelopmental disorders or learning disabilities

The committee thought that that children with neurodevelopmental disorders or learning disabilities should be considered separately because referral to paediatric services may not be appropriate given the prevalence of sleep problems in these children. They agreed that clinical judgement should be used.

Sleep disorders as a result of gastro-oesophageal reflux or constipation

The committee thought it useful to point out that sleep disturbances might also be caused by gastro-oesophageal reflux or constipation, and agreed that the NICE guidelines on these conditions in children would be helpful.

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New-onset slurred or disrupted speech

The committee agreed that new-onset slurred or disrupted speech not attributable to medicines, recreational drugs or alcohol can indicate an acute or progressive neurological disorder or epilepsy, and therefore children with this symptom need urgent neurological assessment.

Problems with speech development in children aged over 2 years

The committee noted that problems with speech development are a very common presentation. They discussed the appropriate age for referral, noting that there is a wide range within which development can be considered normal. They therefore agreed that referrals should not be made before the age of 2 years. Until that age, development may be within normal limits, and speech difficulties may resolve unaided.

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New-onset squint with loss of fundal ('red') reflex

The committee agreed that referral should be immediate as new-onset squint with loss of fundal ('red') reflex in 1 or both eyes can indicate a retinoblastoma, or other progressive pathology of the eye. Additionally, following post publication feedback, NICE felt it was important to highlight that a new-onset squint with loss of fundal ('red') reflex in 1 or both eyes may also indicate a neurological condition associated with raised intracranial pressure or a brain tumour.

New-onset squint with ataxia, vomiting or headache

New-onset squint presenting with accompanying symptoms such as ataxia, vomiting or headache can indicate raised intracranial pressure. The committee agreed that children presenting with squint and any of these symptoms should be referred immediately.

Paralytic squint

Intracerebral tumour or inflammation can present with restriction of movement of 1 or both eyes, so the committee agreed that this symptom should trigger urgent referral.

Non-paralytic squint

In a non-paralytic squint, the child retains the ability to move both eyes fully in all directions. In the absence of any other signs or symptoms such as loss of red reflexes, ataxia, vomiting or headaches, the committee agreed that this symptom warrants a routine referral to ophthalmology.

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Sudden-onset involuntary movements

The committee agreed that sudden onset of chorea, ataxia or dystonia in a child can be a symptom of a progressive neurological disease such as a space-occupying lesion, a metabolic disturbance, a degenerative condition, a para-infectious condition such as rheumatic fever, or drug-induced, so all children with this symptom should be referred immediately.

Tics

The committee agreed that most simple motor tics resolve on their own. Parents or carers often find them concerning and reassurance can be helpful. The committee added that referral might be helpful for children with tics that significantly impair quality of life, because drug treatment or habit reversal therapy can be helpful.

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Tremor of sudden onset or with accompanying neurological signs or symptoms

The committee agreed that children with these symptoms or signs need to be referred for assessment because tremor can be the initial symptom of a space-occupying lesion in children if its onset is sudden or it is accompanied by other neurological signs or symptoms.

Postural tremor

The committee discussed tremor as a side effect of sodium valproate or a beta-adrenergic agonist, or a result of hyperthyroidism, and agreed that these should be considered possible causes of isolated tremor in children taking these medicines.

Because tremor is rarely caused by a progressive neurological condition, the committee thought that effective management of the tremor should be the priority. They noted that occupational therapists have the skills to assess how the tremor affects the child's home and school life.

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