A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

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 [1].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 distinctiv