A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report
© Katsenos et al.; licensee BioMed Central Ltd. 2013
Received: 21 May 2012
Accepted: 2 January 2013
Published: 18 January 2013
Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared. Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolic derangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are: Multiple sclerosis (MS), Acute disseminated encephalomyelitis (ADEM) and Acute hemorrhagic leucoencephalitis. Differentiation is not always easy and there is considerable overlaping. Data about adults with acute demyelination requiring ICU admission is limited.
A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failure requiring mechanical ventilation. She had a history of febrile disease one month before, acute onset of paraplegia, diplopia, progressive arm weakness and dyspnea. Her consciousness was not impaired. A demyelinating central nervous system (CNS) disease, possibly post infectious encephalomyelitis (ADEM) was the underlying condition. The MRI of the brain disclosed diffused expanded cerebral lesions involving the optic nerve, basal ganglia cerebellum, pons and medulla oblongata. There was also extended involvement of the cervical and thoracic part of the spinal cord. CSF leukocyte count was elevated with lymphocyte predominance. The patient required mechanical ventilation for two months. Then she was transferred to a rehabilitation centre. Three years later she remains paraplegic. Since then she has not suffered any other demyelination attack.
Demyelinating diseases can cause acute respiratory failure when the spinal cord is affected. Severe forms of these diseases, making necessary ICU admission, is less frequently reported. Intensivists should be aware of the features of these rare diseases.
The term demyelination describes a destruction of the myelin sheath. Axons that have been demyelinated, secondarily, tend to atrophy and degenerate. Demyelination according to pathogenesis can be classified into several categories: inflammatory, viral, metabolic, hypoxic-ischemic and compressive .
Inflammatory demyelination occurs in multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leucoencephalitis (AHL). MS can be classified, according to clinical and pathological features, into three main subtypes: classical, acute and concentric sclerosis. Devic’s disease (neuromyelitis optica) is correlated with a specific autoantibody (NMO-IgG). It was considered a variant of MS, but recent pathological, immunopathogenic and imaging studies suggest distinctive features . Although there are specific pathological features associated with these diseases, differential diagnosis is mainly based on clinical and medical imaging grounds. In addition, connective tissue diseases, bacterial and viral infections and other conditions, can cause transverse myelopathy.
Excluding Devic’s disease, there is no biological marker that can help differentiate these diseases. Clinical features depend on the region of the CNS that is affected and on the extent of demyelination. Generally, MS is more common in adults, tends to relapse, and is usually monosymptomatic. While ADEM is more common in children, frequently accompanying encephalopathy with seizures. It is not relapsing and follows a history of precipitating infection within two weeks. Optic neuritis is unilateral in MS and bilateral in ADEM. The CSF examination commonly shows oligoclonal IgG in MS and lymphocytosis with increased protein in ADEM, while the MRI shows disseminated inflammatory lesions, which are periventricular or callosal in MS and cortical or in deep gray matter in ADEM.
When the spinal cord is involved in ADEM, lesions are large, swollen and thoracic, whereas in MS they are clearly smaller, more discrete and cervical. The follow up can distinguish the two diseases. In ADEM new lesions should not occur and original lesions are expected to resolve partially or completely. If a new lesion occurs after the acute/subacute phase, a diagnosis of MS is more probable. In many cases, however, the final diagnosis can only be made after a considerably long period and follow up examinations [3–7]. Although ADEM in adults has been studied, there is limited data of severe forms requiring ICU admission. Common causes for referral to ICU are seizures, impaired consciousness and fever. Prognosis is poor and survivors have functional impairments .
A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failure requiring mechanical ventilation. A degenerative central nervous system (CNS) disease, possibly post infectious encephalomyelitis (ADEM) was the underlying condition. Upon hospital admission the patient complained of headache, low back pain and fever. Within days, the patient progressively deteriorated with muscle weakness in the upper and lower extremities.
Three years after discharge the patient is still paraplegic despite the resolution of the lesions in MRI. She complains of a bilateral loss in her senses of heat and touch at the level of the thighs.
Differential diagnosis between demyelinating diseases is not easy due to a lack of specific biological markers. In our case, the history of a febrile disease a month before the development of neurological symptoms supports the diagnosis of ADEM. Our patient did not suffer any demyelination attack during the three years of follow up. This is also supportive to the diagnosis of ADEM, which is monophasic illness as opposed to relapsing-remitting MS. Contrary to this diagnosis is the fact that patient’s consciousness remained intact while she had no seizures. Devic’s disease was ruled out by the absence of the NMO-IgG.
A possible cause of the acute respiratory failure is the extensive spinal cord involvement, as the patient under clinical examination displayed prolonged intercostal muscle weakness. Unfortunately respiratory function testing was not done before intubation and the respiratory failure was not well studied.
A persistent paraplegia along with a loss of senses in heat and touch despite MRI normalisation is another interesting point worthy of observation. One possible explanation is the development of critical illness polyneuropathy (CIP) and myopathy (CIM) during the prolonged ICU stay. These two clinical entities may occur concomitantly and are associated with sepsis, multiorgan failure and possibly with corticosteroid treatment .
Our patient was treated with high doses of corticosteroids and suffered many times from sepsis and severe sepsis during her long ICU hospitalisation. Unfortunately, she was not available for nerve conduction studies and electromyography.
Another explanation is that the traditional MRI in MS and in other demyelinating diseases has a poor correlation with clinical disability. The MRI can detect changes in inflammatory activity but for quantification of intact myelin and remyelination, magnetization transfer imaging has been proposed. Diffusion tensor imaging can be used to monitor tract-specific changes that may be more closely linked to clinical measures and may be particularly powerful when combined with functional measures, such as functional MRI [10, 11].
Severe forms of demyelinating diseases, requiring ICU admission, bear a poor prognosis. Clinical recovery may be limited despite resolution of imaging changes.
“Written informed consent was obtained from the patient, who is now 20 years old, for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.”
Magnetic resonance imaging
Acute disseminated encephalomyelitis
Angiotensin converting enzyme
Critical illness polyneuropathy
Critical illness myopathy.
The authors acknowledge the patient for her kind permission to use her medical file for this publication.
The authors have no financial funding to disclose for this publication.
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