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ADMISSION TO NEUROLOGICAL
INTENSIVE CARE: WHO, WHEN,
*
iii2
AND WHY?
Robin S Howard, Dimitri M Kullmann, Nicholas P Hirsch
J Neurol Neurosurg Psychiatry 2003;74(Suppl III):iii2–iii9

T
he majority of neurologists work in district general or teaching hospitals with large general
intensive care units (ICUs). In this setting, ICUs require an increasing input from neurologists,
especially with regard to the assessment of hypoxic brain damage and the neurological com-
plications of organ failure, critical illness, and sepsis. In contrast, dedicated neurological intensive
care units (NICUs) tend to deal largely with a different population of patients. Such units are pri-
marily concerned with the management of primary encephalopathic patients, the control of raised
intracranial pressure (ICP), the management of ventilatory, autonomic, and bulbar insufficiency,
and the consequences of profound neuromuscular weakness. This role encompasses the treatment
of mechanical ventilatory failure, specific treatments (both medical and surgical) and general
medical complications of these disorders.1
In general, NICU patients with primary neurological diseases such as myasthenia gravis,
Guillain-Barré syndrome, central nervous system infections, status epilepticus, and stroke have a
better outcome than those patients with secondary neurological disease seen on general ICUs.
However, such patients remain dependent on ICU support for very much longer periods of time.
This results in very significant psychological demands on the patients, their carers, the nurses,
physicians, and other health care professionals. In this review we will consider the rationale for
managing acute neurological conditions in a dedicated NICU environment.

c INDICATIONS FOR ADMISSION

Indications for admission to NICU include:

c impaired level of consciousness
c impaired airway protection
c progressive respiratory impairment or the need for mechanical ventilation (box 1)
c seizures
c clinical or computed tomographic (CT) evidence of raised ICP caused by a space occupying

lesion, cerebral oedema or haemorrhagic conversion of a cerebral infarct

c general medical complications (for example, hyper/hypotension, aspiration pneumonia, sepsis,

cardiac arrhythmias, pulmonary emboli)

c monitoring (for example, level of consciousness, respiratory function, ICP, continuous electroen-

cephalography (EEG))
c specific treatments (for example, neurosurgical intervention, intravenous or arterial thromboly-

sis).
Before examining these groups in more detail it is essential to emphasise that there are general
principles of intensive care management common to all units, whatever their specialisation. These
include meticulous nursing and medical care and, crucially for our patients, physiotherapy. Early
and aggressive physiotherapy intervention (including frequent alterations of limb positioning,
passive limb movements, and appropriate splinting) helps to maintain joint mobility and prevents
limb contractures and pain while awaiting neurological improvement.
Other aspects of general ICU care include the management of agitation and pain, maintenance
of an adequate airway and ventilation, cardiovascular stability, nutrition, anticoagulation, throm-
bolysis, and raised ICP; these will be discussed in related articles.
Many patients with impaired consciousness or severe neuromuscular weakness are not able to
See end of article for authors’ communicate adequately. It is essential that satisfactory means of communication are established
affiliations as soon as possible. Furthermore, when communication is difficult, the family often represents the
patient’s interests and they must therefore have access to medical and nursing staff throughout so
Correspondence to: that they understand the immediate clinical situation, management, and outlook.
Dr Robin Howard, The Batten
Harris Medical Intensive Care The central and peripheral causes in ventilatory insufficiency or failure which may require
Unit, The National Hospital for admission to the NICU are listed in tables 1 and 2.
Neurology and Neurosurgery,
Queen Square, London Stroke
WC1N 3BG, UK;
[email protected] In contrast to other European countries, admission to an NICU following stroke is relatively
uncommon in the UK. However, patients with acute stroke, whether haemorrhagic, ischaemic or

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NEUROLOGY IN PRACTICE

Box 1: Neurological indications for mechanical

ventilation
c Failure of central regulation of respiration (apnoea, ataxic or
cluster breathing)
c Inability to protect airway
c

c
Brain swelling with depressed level of consciousness (Glas-
gow coma score < 9)
Impending neuromuscular respiratory failure (forced vital
*
iii3
capacity < 20 ml/kg, tachypnoea, dyspnoea at rest, use of
accessory muscles, staccato speech)
Respiratory failure must be anticipated before the emergence
of hypoxia and/or hypercapnia. Thus the threshold for intuba-
tion is lower in the context of rapidly progressive neuromusc-
ular weakness.

venous, require resuscitation and close monitoring in an

attempt to prevent the secondary cerebral insults and the sub-
sequent clinical deterioration that results from major systemic
derangements. This is best achieved in an intensive care or
high dependency environment where physiological monitor-
ing can be efficiently undertaken. Obviously not all stroke
sufferers can be admitted to a specialist ICU and the level of
care will depend on the availability of local stroke units and
the condition and prognosis of the patient.
The principles of assessment and resuscitation from acute
stroke are similar regardless of the underlying cause and Figure 1 Axial T1 weighted MRI scans showing evolution of
include: middle cerebral artery occlusion causing extensive infarction with
c airway management—tracheal intubation is indicated mass effect. The appearances after decompressive craniotomy are
when there is: shown in the third panel.
– impaired level of consciousness (for example, Glasgow
coma score < 9) horizontal and vertical distortion and shift of the brainstem.
– progressive respiratory impairment or respiratory failure This change in dynamics may not always being reflected by
– impaired cough and airway clearance ICP measurements . The management of this brain swelling is
– pulmonary oedema/aspiration often problematic (see Dunn in issue 74). Osmotic diuretics
– seizure activity and hyperventilation are rarely effective and some advocate
– intubation may also be required before diagnostic or
decompressive craniectomy if extensive cerebral swelling
therapeutic procedures such as magnetic resonance
occurs, especially following non-dominant infarcts.5 Other
imaging (MRI) or thrombolysis
c maintenance of adequate arterial blood pressure/cerebral
causes of deterioration include haemorrhagic transformation
of the infarct, which may produce diencephalic brain hernia-
perfusion pressure
c intravenous fluid management tion, the development of seizures, and systemic factors
c temperature control including congestive cardiac failure, pulmonary oedema,
c control of seizures cardiac arrhythmias or pulmonary emboli.
c institution of enteral nutrition
c ICP management Acute basilar occlusion
c medical treatment of complications (for example, sepsis)
Acute basilar occlusion (fig 2) is a potentially fatal event
c other management related to the underlying cause (for requiring early recognition and urgent intervention. Prognosis
example, anticoagulation, thrombolysis, evacuation of hae- is particularly poor if there is rapid progression to coma with
matoma, clipping and coiling of intracerebral aneurysms). the need for tracheal intubation and ventilatory support.
Because of the devastating consequences of established basilar
Middle cerebral artery occlusion occlusion, aggressive intervention may be indicated in the
There is much debate surrounding aggressive therapeutic early stages. Anticoagulation with intravenous heparin re-
intervention in patients with extensive infarction caused by mains the mainstay of treatment, but thrombolysis up to 12
acute middle cerebral artery occlusion (fig 1) in whom expec- hours after onset or continuing progression may be indicated
tation for functional recovery is low.2 3 Following resuscitation despite the risks of fatal intracranial haemorrhage (although
and stabilisation there is evidence for early and aggressive randomised trial data are lacking). Late deterioration occurs in
intervention with thrombolysis. Numerous exclusion criteria up to a third either because of extension of the thrombus
(including > 3 hours elapsed from stroke onset and wide- causing successive occlusion of the perforating arteries or due
spread early infarct changes on CT scan) currently mean that to “artery-to-artery” emboli arising distally from the site of
alteplase is probably only appropriate for a small percentage of the occluded vessel. The prognosis is usually poor in patients
patients reaching the NICU. Clinical deterioration following who present with established infarction, but those with
middle cerebral artery infarction is common and associated limited infarcts of the pons may have a reasonable functional
with cerebral oedema, usually developing between 2–7 days. outcome, especially with early thrombolysis or anti-
Oedema and infarction causes a mass effect leading to coagulation, particularly if admitted and stabilised before the

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NEUROLOGY IN PRACTICE

Supratentorial intracerebral haemorrhage (ganglionic

or lobular)
Admission to the NICU will be determined by the clinical state
of the patient and the prognosis. Following adequate resusci-
tation there remains considerable uncertainty about the role
of surgical intervention (for example, evacuation of hae-

*
iii4 matomata, drainage of hydrocephalus, decompressive craniec-
tomy), but aggressive treatment may be indicated when
monitoring reveals raised ICP unresponsive to medical
treatment. Acute deterioration may occur in 30–60% of
patients and usually within the first two days; close monitor-
ing is therefore essential. The causes of deterioration include
increase in haematoma volume, development of penumbral
oedema, obstructive hydrocephalus, or systemic complications
such as aspiration pneumonia, sepsis or cardiac arrhythmias.

Infratentorial intracerebral haemorrhage (cerebellar or

brainstem)
The acute management will usually involve early tracheal
intubation and mechanical ventilation and urgent control of
ICP. Clinical deterioration is common and is usually secondary
Figure 2 Axial diffusion weighted MRI scan showing extensive to direct brainstem compression accompanied by cerebellar
brainstem infarction in a patient with basilar occlusion associated
herniation rather than obstructive hydrocephalus. Rebleeding
with antiphospholipid syndrome and dehydration.
is a neurosurgical emergency and the high mortality often
justifies surgical evacuation.
brainstem dysfunction is fully established or mechanical ven- Cerebral venous thrombosis
tilation required. Cerebral venous thrombosis is particularly important to
recognise. Although robust randomised trial data are lacking,
Cerebellar infarcts there is general consensus that early anticoagulation can
Cerebellar infarcts may be difficult to recognise on account of result in good clinical outcome, even in the face of
the slow evolution of brainstem and cerebellar signs. Late haemorrhagic venous infarction. MR and CT vascular imaging
deterioration is associated with an increase in infarct volume has made it easier to establish the diagnosis, but close moni-
leading to brainstem involvement and herniation, compres- toring of the patient is essential as late deterioration can have
sion of the brainstem caused by oedema, and the development many causes. These include extension of the thrombosis,
of hydrocephalus resulting from obstruction of cerebrospinal development of haemorrhagic infarction, raised ICP second-
fluid flow. ary to cerebral oedema, seizures, the development of systemic
complications including aspiration pneumonia, pulmonary
Subarachnoid haemorrhage emboli, and sepsis, and complications of an often associated
Admission to NICU is indicated, where possible, for all hypercoagulable state.
patients with subarachnoid haemorrhage, to manage systemic
complications, recognise and treat clinical deterioration, Status epilepticus
investigate the cause of the haemorrhage, and to treat any Patients with severe epilepsy and status epilepticus are often
underlying aneurysm or arteriovenous malformation. Resus- admitted to general intensive care units. Commonly identified
citation is directed towards maintaining cerebral perfusion causes of status are medication change, encephalitis, trauma,
pressure by providing adequate arterial blood pressure (often cerebrovascular disease, tumours or acute metabolic or toxic
with the use of inotropes to produce relative hypertension), disturbances. However, NICUs will often have to treat patients
ensuring a relatively high circulating blood volume (hypervol- with known epilepsy who have developed refractory status,
aemia), and producing relative haemodilution (“triple H which has not responded to conventional management either
therapy”). Other aspects of management in the acute stages because of severe underlying epilepsy or serious cerebral irri-
include suitable analgesia, seizure control, and treatment with tation caused by the underlying cause.
nimodipine to prevent secondary ischaemia caused by Following adequate resuscitation the treatment of status
vasospasm. Angiography and definitive treatment should be epilepticus in the NICU proceeds simultaneously on four
undertaken as soon as possible. Although the relative role and fronts: termination of seizures, prevention of seizure recur-
timing of operative clipping and endovascular treatments are rence once status is controlled, management of the precipitat-
still uncertain, it is clear that these patients should be ing causes, and management of the complications. The drug
monitored in an intensive care environment. Sudden death treatment of status epilepticus has been reviewed recently7–9
may occur in up to 10% of cases because of early rebleeding, and admission to NICU should be undertaken as soon as pos-
intraventricular extension of the haemorrhage, or general sible when it is clear either that conventional treatment (that
medical complications including pulmonary aspiration, car- is, lorazepam, phenytoin, phenobarbitone) has failed to abort
diac arrhythmias, and neurogenic pulmonary oedema. Clinical seizure activity, or if there is significant sedation, failure to
deterioration may also develop because of delayed cerebral protect the airway or ventilatory impairment, or when general
ischaemia caused by progressive vasospasm, enlargement of anaesthesia is indicated. In addition to monitoring respiratory
intracerebral haematoma or the development of and cardiac function, continuous EEG monitoring is necessary
hydrocephalus.6 in prolonged and refractory status. The appropriate titration of

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NEUROLOGY IN PRACTICE

Acute bacterial meningitis

Table 1 Central causes of ventilatory insufficiency or Bacterial meningitis remains a potentially devastating neuro-
failure which may require admission to the NICU
logical disorder. Although survival rates have improved, the
c Cortical Epilepsy mortality rate of acute meningitis remains significant and
Vascular there is a high incidence of residual severe neurological deficit,
Tumour
particularly if there is a delay in initiating treatment and
c Brainstem Congenital (Ondine’s curse)—primary alveolar
hypoventilation
Tumour
monitoring for complications.11 Adult meningitis is commonly
caused by Streptococcus pneumoniae (pneumococcus) or Neisseria *
iii5
Vascular meningitides (meningococcus). Escherichia coli and Staphylococ-
Multiple sclerosis and acute disseminated cus species account for a small number of cases while Haemo-
encephalomyelitis philus influenzae occurs in children. Less commonly meningitis
Motor neurone disease
Infection: Borrelia may be caused by Listeria monocytogenes and Pseudomonas
Listeria species, especially in the immunocompromised patient. Tuber-
Post-varicella encephalomyelitis culous meningitis may present at any age, and requires a high
Poliomyelitis
index of suspicion. Patients with meningitis are usually
Encephalitis lethargica
Western equine encephalitis admitted to an ICU when in coma, when there are complica-
Paraneoplastic tions such as seizures and cerebral oedema, or because they
Leigh’s disease have developed systemic problems including septicaemia, pul-
Reye’s syndrome
Hypoxaemia monary aspiration or cardiopulmonary compromise. The ICU
environment allows close supervision of supportive and
c Formen magnum and upper cervical cord specific treatment.
Arnold Chiari malformation – cerebellar ectopia
Achondroplasia, osteogenesis imperfecta
It is essential to exclude cerebral abscess and subdural or
Rheumatoid arthritis—odontoid peg compression extradural empyema as well as other causes of meningism
Trauma with appropriate imaging before lumbar puncture is under-
Vascular
taken. If imaging facilities are unavailable, urgent empirical
c Disorders of the spinal cord treatment with ceftriaxone or cefotaxime is indicated. The
Acute epidural compression due to neoplasm or early complications of acute meningitis include the develop-
infection ment of cerebral oedema, transtentorial herniation, and coma.
Acute transverse myelitis
Cord infarction Immediate transfer to an ICU is necessary under these
Other myelopathies (including traumatic) circumstances. Bacterial meningitis caused by any organism
Tetanus may result in septic or hypovolaemic shock. Thus, haemody-
c Autonomic Multi system atrophy
namic and respiratory monitoring are mandatory at an early
stage and mechanical ventilation may be required if there is
c Extrapyramidal Status dystonicus evidence of cardiorespiratory compromise. Bacterial meningi-
tis is a notifiable disease. Chemoprophylaxis with rifampicin
or ciprofloxacin is indicated for all household and healthcare
contacts of patients with meningococcal infection.
In adults with normal renal function the recommended
empirical treatment for bacterial meningitis consists of intra-
anaesthetic agents during status epilepticus may be based on
venous (iv) ceftriaxone 4 g followed 24 hours later by 2 g iv
the appearance of burst suppression on the EEG. Furthermore
daily, cefotaxime 2 g iv every four hours, benzylpenicillin 1.2 g
continuous recording will give an indication of worsening of
iv every four hours, or ampicillin 2 g iv every four hours, all of
generalised convulsive status epilepticus regardless of the which cover the common pathogens and most enteric Gram
presence or absence of sedating drugs or paralysing agents. It negative organisms. If resistant pneumococcus or listeria is
is striking that in a relatively recent survey less than a third of suspected or there is a concern about an immunosuppressed
units monitored status by continuous EEG or cerebral patient, vancomycin 2 g iv every 12 hours should be added. If
function monitor, and almost a half used clinical monitoring patients have a penicillin allergy chloramphenicol 2 g every six
only.10 hours is recommended.
The complications of status epilepticus relate either to the Where tuberculous meningitis is suspected treatment
cerebral and metabolic consequences of prolonged seizures or should be instituted, and may need to be continued without
the effects of medical treatment. Cardiopulmonary problems positive bacteriological evidence for several weeks until the
include the development of aspiration pneumonia, adult results of cultures are negative. Standard antituberculous
respiratory distress syndrome, pulmonary emboli, myocardial treatment consists of isoniazid (20 mg/kg once daily),
ischaemia, and cardiac arrhythmia. Hyperthermia is common rifampicin (20 mg/kg) and pyrazinamide (40 mg/kg), together
and rhabdomyolysis may develop. Prolonged hypoxia may with pyridoxine (10 mg). Second line drugs (streptomycin,
cause cerebral damage and electrolyte disturbance, and meta- ethionamide, and ethambutol) may be required to treat resist-
bolic acidosis may contribute to the development of multi- ant organisms.
organ failure. Many drug treatments used in status epilepticus The role of steroids in the treatment of adult bacterial men-
cause sedation, respiratory depression, and hypotension. Arti- ingitis continues to be debated. A recent trial lent support to
ficial ventilation is required if general anaesthesia is indicated the use of dexamethasone (10 mg every six hours, started
or if the seizures remain difficult to control. It is also necessary with the first dose of antibiotics) for pneumococcal meningi-
to maintain systemic blood pressure at normal or supranormal tis. Weaker evidence supports its adjuvant use in tuberculous
levels to ensure adequate cerebral perfusion. Fluid resuscita- meningitis.
tion and/or inotropic support should be guided by appropriate Late deterioration following acute bacterial meningitis may
cardiovascular monitoring. be caused by antibiotic resistance or to the development of

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NEUROLOGY IN PRACTICE

The use of polymerase chain reaction for the diagnosis of

Table 2 Peripheral causes of ventilatory insufficiency
herpes simplex encephalitis14 and other causes of viral
or failure which may require admission to NICU
encephalitis (for example, cytomegalovirus (CMV), Epstein-
c Anterior horn Motor neurone disease Barr virus, enterovirus) has meant that brain biopsy is usually
cell Poliomyelitis or post-polio syndromes
Rabies
no longer necessary to reach a definitive diagnosis. The man-

*
agement of acute viral encephalitis includes appropriate
c Multiple Carcinomatous meningitis airway management, fluid and nutritional support, and treat-
iii6 radiculopathies AIDS polyradiculitis
ment of confusion and seizures. Specific treatment for herpes
c Polyneuropathy Acute inflammatory demyelinating polyneuropathy simplex encephalitis with aciclovir (10 mg/kg iv every eight
(AIDP) hours for at least 10 days) reduces mortality by 25%, particu-
Acute motor and sensory axonal neuropathy
(AMSAN)
larly in the most severely affected, with up to 40% of such
Acute motor axonal neuropathy (AMAN) patients making a good or complete recovery.15 For CMV
Critical illness polyneuropathy encephalitis ganciclovir is indicated (10 mg/kg every 12
Other polyneuropathies:
hours).
Hereditary motor-sensory
Acute porphyria Clinical deterioration in herpes simplex encephalitis is usu-
Organophosphate poisoning ally the result of severe cerebral oedema with diencephalic
Herpes zoster/varicella herniation or systemic complications, including generalised
Neuralgic amyotrophy
sepsis and aspiration (fig 3). Furthermore, progressive
c Neuromuscular Myasthenia gravis worsening of focal seizures may lead to status epilepticus. The
transmission Lambert-Eaton myasthenic syndrome use of ICP monitoring in acute encephalitis remains contro-
defects Neuromuscular blocking agents
Other:
Botulism
versial but should be considered if there is a rapid
Toxins deterioration in the level of consciousness, and imaging
Hypermagnesaemia suggests raised ICP. In this situation, aggressive treatment
Organophosphate poisoning
including tracheal intubation and mechanical ventilation with
c Muscle Dystrophy—Duchenne, Becker, limb girdle, Emery appropriate sedation should be instituted, and seizures
Dryfuss treated. Prolonged sedation or general anaesthesia may be
Inflammatory
necessary. Decompressive craniotomy may be successful in
Myotonic dystrophy
Metabolic: cases where there is rapid swelling of a non-dominant tempo-
Acid maltase deficiency ral lobe as poor outcome is likely without.
Mitochondrial myopathies
Myopathies associated with neuromuscular Acute parainfectious inflammatory encephalopathy
blocking agents and steroids
Acute quadriplegic myopathy
Acute disseminated encephalomyelitis (ADEM) and acute
Myopathy and sepsis haemorrhagic leucoencephalitis (AHL) are inflammatory dis-
Cachectic myopathy orders presumed to be the product of an autoimmune
HIV related myopathy
response to infection, although a clear history of preceding ill-
Sarcoid myopathy
Hypokalaemic myopathy ness is often absent.16 In AHL and the more aggressive presen-
Rhabdomyolysis tation of ADEM, patients may develop rapid and profound
Periodic paralysis deterioration in the level of consciousness and present in coma
or with established focal neurological deficits, causing
ventilatory impairment or failure to protect the airway.

cerebral oedema, subdural effusion or empyema, superior sag-

ittal sinus thrombosis, hydrocephalus, the development of
focal neurological signs caused by an associated vasculitis, or
systemic complications including pericardial effusion and
polyarteritis.

Acute viral encephalitis

Acute infectious encephalitis and parainfectious inflamma-
tory encephalopathy are rare complications of common viral
infections. A causal agent is found in less than 50% of cases of
viral encephalitis.12 13 The most common cause is herpes
simplex virus but other human herpesviruses (for example,
varicella zoster), enterovirus, mumps, measles, and viruses
associated with respiratory tract infections (adenovirus and
influenza B) are also important causes. Encephalitis caused by
HIV or related to immunosuppression in AIDS is becoming
increasingly frequent.
Viral encephalitis may present with the rapid development
of encephalopathy and coma; this often requires tracheal
intubation for airway protection and ventilatory support, con-
trol of raised ICP, and the effective treatment of seizures.
Patients are often confused, restless or aggressive, even in the Figure 3 Coronal T1 weighted MRI showing extensive grey and
absence of focal neurological signs, and sedation may be nec- white matter changes with a temporal lobe emphasis in a patient
essary. with herpes simplex encephalitis.

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NEUROLOGY IN PRACTICE

Although high doses of corticosteroids are commonly given failure or acute bulbar (or limb) weakness. The underlying
there is no randomised controlled trial evidence for their effi- neuromuscular disease may be a primary presentation (for
cacy, and mortality from AHL is high even if patients are example, Guillain-Barré syndrome) but may also have been
promptly admitted to the NICU. The prognosis for ADEM is relatively stable (for example, post-poliomyelitis), slowly pro-
rather better and a proportion of patients may make a good gressive (for example, acid maltase deficiency), more rapidly
recovery, although many are left with significant residual cog-

*
progressive (for example, motor neurone disease or Duchenne
nitive or focal deficits. muscular dystrophy) or relapsing or intermittent (for exam-
ple, myasthenia gravis). Patients with these disorders may
iii7
Multiple sclerosis
develop severe and unexpected deterioration of respiratory,
Patients with multiple sclerosis may develop respiratory
bulbar, and limb function as a consequence of intercurrent
insufficiency.17 This is multifactorial and may be associated
events including systemic infection, disease exacerbation, and
with respiratory muscle weakness, bulbar dysfunction, and
increased pulmonary load—for example, pregnancy or obes-
disorders of the regulation of breathing. Acute multiple
ity. It must also be emphasised that patients may develop neu-
sclerosis relapses caused by plaques in the brainstem or high
ropathy, myopathy or disorders of neuromuscular transmis-
cervical cord may lead to acute respiratory and bulbar failure.
Admission to the NICU and temporary airway protection and sion as a consequence of multiorgan failure, sepsis or
respiratory support, either continuous or confined to the prolonged ICU care. These conditions are considered else-
period during sleep, may be necessary during acute bulbar or where in this issue.
spinal relapses. The presence of coexisting bulbar weakness may result in a
failure to clear retained secretions leading to pulmonary aspi-
Autonomic neuropathy/movement disorders ration and the development of bronchopneumonia. Thus
Patients with autonomic neuropathy, especially those associ- patients with neuromuscular disease must be closely moni-
ated with parkinsonian syndromes (for example, multisystem tored for the development of a progressive decline in forced
atrophy) may develop respiratory insufficiency related to vital capacity, arterial oxygen saturation, and arterial blood
impairment of the control of breathing and vocal cord paresis. gas tensions. Regular chest x rays are often indicated.
Admission to the NICU for ventilatory support, either
intermittent or continuous, is occasionally necessary. Move- Acute poliomyelitis/motor neurone disease/
ment disorders, in particular status dystonicus, may require mitochondrial disease
admission for respiratory or bulbar compromise or physical During acute poliomyelitis respiratory insufficiency occurs as
exhaustion.18 a result of respiratory muscle weakness or involvement of the
central respiratory control mechanisms. Respiratory insuffi-
Cervical spinal cord lesions ciency may develop many years after poliomyelitis, even in the
Lesions of the cervical spinal cord may lead to acute absence of any obvious respiratory involvement during the
respiratory failure requiring ventilatory support; these condi- acute illness or convalescent phase.
tions include postinflammatory myelitis, structural lesions Respiratory insufficiency is the common terminal event in
caused by rheumatoid disease, and other skeletal abnormali- motor neurone disease either due to respiratory muscle or
ties at the foramen magnum. Acute deterioration is often pre- bulbar weakness leading to alveolar hypoventilation, pulmo-
cipitated by trauma or intercurrent events (fig 4). nary aspiration, bronchopneumonia or pulmonary emboli.
However, some patients with motor neurone disease may
Neuromuscular disease
Patients with neuromuscular disease will require care in an develop respiratory insufficiency early in the course of the dis-
NICU if they develop acute or acute on chronic respiratory ease and may even present with respiratory failure or respira-
tory arrest. Such patients may undergo tracheal intubation
Figure 4 Sagittal T2 weighted and mechanical ventilation, and be admitted to general ICUs
MRI scan showing cord infarction before the diagnosis has been made. The progressive nature of
causing respiratory failure the condition means that weaning to non-invasive ventilatory
caused by anterior spinal artery support may be impossible and requires the specialist support
occlusion. of a NICU.
Mitochondrial disease may present to the NICU as status
epilepticus, with recurrent stroke-like events, as a metabolic
coma caused by unexplained lactic acidosis, or with respira-
tory failure caused by central or peripheral impairment of
ventilation.19

Guillain-Barré syndrome
The role of the ICU in the management of Guillain-Barré syn-
drome is well documented. The indications for admission
include ventilatory insufficiency, severe bulbar weakness
threatening pulmonary aspiration, autonomic instability, or
coexisting general medical factors. Often a combination of
factors is present. The incidence of respiratory failure
requiring mechanical ventilation in Guillain-Barré syndrome
is approximately 30%. Ventilatory failure is primarily caused
by inspiratory muscle weakness, although weakness of the
abdominal and accessory muscles of respiration, retained air-
way secretions leading to pulmonary aspiration and atelecta-
sis are all contributory factors.20 21 The associated bulbar

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NEUROLOGY IN PRACTICE

weakness and autonomic instability reinforce the need for weakness. Patients with recent onset generalised myasthenia
control of the airway and ventilation. Acute motor and sensory gravis started on a high dose, daily corticosteroid regimen are
axonal neuropathy, the acute axonal form of Guillain-Barré particularly at risk for acute paradoxical deterioration during
syndrome, usually presents with a rapidly developing paraly- the first 48–96 hours of treatment. Thymectomy should be
sis developing over hours and the rapid development of respi- coordinated by an NICU with experience of the procedure;

*
ratory failure requiring tracheal intubation and ventilation. postoperative management requires such experience.
iii8 There may be total paralysis of all voluntary muscles of the A number of primary myopathic disorders may present with
body, including the cranial musculature, the ocular muscles, acute ventilatory failure, but this usually develops when there
and the pupils. Prolonged paralysis and incomplete recovery has been preceding hyoventilation and these conditions will
are more likely and prolonged ventilatory support may be be discussed elsewhere.
necessary.
Using Guillain-Barré syndrome as an example of the prob- Botulism
lems of the long term paralysed and ventilated patient the Botulism must be distinguished from Guillain-Barré syn-
more common complications include: drome and myasthenia gravis. Patients with acute bulbar and
c Respiratory tract infections that may be associated with aspira- respiratory impairment require admission to the NICU. There
tion caused by oropharyngeal weakness but are also may be blurred vision, diplopia, ptosis, dysarthria, dysphagia,
common in the paralysed patient as a consequence of noso- and progressive descending limb weakness. Mechanical venti-
comial infections associated with endotracheal tubes or tra- lation is often required. Prominent gastrointestinal and auto-
cheostomy. nomic symptoms in addition to weakness are indicative of
c Cardiac involvement characterised by arrhythmias caused by botulism, but definitive diagnosis requires detection of toxin
autonomic impairment, most often tachycardias but more in the patient’s serum, stool or food. Treatment consists
seriously bradyarryhthmias. primarily of supportive care. Antitoxin may be helpful,
c As with other acute neurological disorders requiring venti- particularly in type E botulism, but in most adult patients
lation, mild hyponatraemia is common and usually associ- treatment with antitoxin does not lead to significant improve-
ated with the syndrome of inappropriate antidiuretic ment. If patients survive the acute phase of illness, recovery is
hormone secretion. usually complete. Mortality in the era of the ICU is less than
c Gastrointestinal involvement may include the development of
10%.
paralytic ileus, which may be severe.
c Confusion is also particularly frequent in ventilated patients Tetanus
and often unrecognised. Most patients with tetanus will be admitted to an NICU
Following recovery most patients report that hallucinations, because of increased muscle tone and spasms which typically
often distressing, occurred during the period of ventilation. begin in the masseter muscles, resulting in the classic finding
The abnormal mental state is multifactorial, probably relating of trismus. Respiratory compromise is caused by spasm of res-
to metabolic and acid base disturbances, drugs, pain, and sen- piratory muscles or laryngospasm. Autonomic dysfunction
sory deprivation. Pain is particularly common in Guillain- occurs in severe cases and results in heart rate and blood pres-
Barré syndrome (more than half of patients during admis- sure lability, arrhythmias, fever, profuse sweating, peripheral
sion) but is also a major problem in other acute neurological vasoconstriction, and ileus. Muscle rupture and rhabdomyoly-
disorders. sis can complicate extreme cases. Treatment of tetanus
The neurocritical care unit is also the most appropriate place includes removal of the source of the toxin, through wound
for the provision of immunotherapy—particularly plasma cleaning and debridement. Human tetanus immunoglobulin
exchange and possibly intravenous human immuno- neutralises circulating toxin but has no effect once the toxin is
globulins—and the reduction in morbidity and mortality neural bound, and should therefore be administered early.
associated with these treatments is likely to be partly related Supportive care consists of treatment in a quiet ICU setting to
to the specialised ICU environment in which the treatments allow cardiorespiratory monitoring with minimal stimulation.
are usually given. Benzodiazepines are used to control muscle spasm and large
Acute ventilatory failure requiring admission to the NICU, doses may be required. Therapeutic paralysis with neuromusc-
intubation, and ventilation may be caused by phrenic ular blocking agents may be necessary in severe cases. Intuba-
neuropathies which will be discussed elsewhere in this issue. tion may be required owing to hypoventilation caused by
muscular rigidity or laryngospasm and should be performed
Myasthenia gravis in a controlled, elective manner if possible. Profound muscle
In myasthenia gravis admission to the NICU is indicated by rigidity increases energy needs and insensible fluid losses, and
the development of incipient ventilatory failure, progressive additional hydration and nutritional supplementation are
bulbar weakness leading to failure of airway protection, or required. Treatment in the ICU has resulted in a pronounced
severe limb and truncal weakness causing extensive paralysis. improvement in prognosis for patients with tetanus. Modern
Admission should be determined by the rate of progression, mortality is approximately 10%. Severe muscular rigidity may
the presence of bulbar weakness, and the clinical state of the last for weeks, with mechanical ventilation required for up to
patient rather than an absolute level of forced vital capacity 3–4 weeks. A complete recovery is typical, although mild pain-
alone. Respiratory failure often results from a myasthenic cri- ful spasms can persist for months.
sis (usually precipitated by infection, surgery or inadequate
treatment), but may also more rarely be precipitated by a THE ROLE OF THE NEUROLOGICAL ICU
cholinergic crisis. The associated bulbar weakness predisposes The primary role of the NICU is that of the management of
to pulmonary aspiration and acute respiratory failure necessi- acute neurological emergencies. This involves control of
tating urgent tracheal intubation and ventilation. Expiratory airway, respiratory, bulbar, and haemodynamic compromise. It
and inspiratory intercostal and diaphragm weakness is also entails the provision of specific treatments and the
common even when there is only mild peripheral muscle prevention and management of the secondary complications.

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 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.74.suppl_3.iii2 on 21 August 2003. Downloaded from http://jnnp.bmj.com/ on July 17, 2022 by guest. Protected by copyright.
NEUROLOGY IN PRACTICE

application of these techniques to support and treat patients

Abbreviations
who are critically ill. The aim is to prevent unnecessary
ADEM: acute disseminated encephalomyelitis morbidity and mortality while maintaining the pastoral
AHL: acute haemorrhagic leucoencephalitis aspects of care.
CMV: cytomegalovirus
CT: computed tomography .....................
EEG: electroencephalogram
ICP: intracranial pressure
ICU: intensive care unit
Authors’ affiliations
R S Howard, D M Kullmann, N P Hirsch, The Batten Harris Medical
Intensive Care Unit, The National Hospital for Neurology and
*
iii9
Neurosurgery, London, UK
MRI: magnetic resonance image
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