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.
The MRI of the brain was normal (Figure 1A), but the cervical and upper thoracic MRI revealed intramedullary inflammatory lesions extending from C3 to Th5 (Figure 1B). The patient was treated with high doses of corticosteroids (methylprednisolone 1gr per day for 4 days), with no response. Gradually, visual disturbances developed and the patient complained of dyspnea. An oxygen saturation of 85% was observed by pulse oxymetry, and oxygen administration (Venturi mask 50%) followed. Her condition rapidly deteriorated. The patient was intubated immediately, supported with mechanical ventilation and transferred to the ICU. (No spirometric evaluation or blood gas analysis was performed).
Intravenous immune globulin (IVIG) treatment was administered (30 grams per day for 4 days). An MRI of the brain revealed diffused extensive lesions, mainly in brain stem and cerebellum, with a high T2 signal compatible with myelitis (Figure 2A). The MRI of the spine revealed extensive confluent lesions extending cranially up to the medulla oblongata and caudally to the middle of the thoracic spine (Figure 2B). CSF was abnormal (WBC = 240/ml, mainly lymphocytes, total protein = 750 mg/dl, glucose ratio CSF/serum = 45/92 mg/dl and no oligoclonal bands of IgG). CSF PCR examination was negative for HSV1,2, enteroviruses, n. meningitis, streptococcus pneumonia, listeria, haemophilus influenza, TBC. In the ICU no additional data was found by repeated cerebral MRI. Neutropenia developed and bone marrow examination was performed, which disclosed a normal bone marrow. The neutropenia probably was due to neutrophil's peripheral destruction, as the patient suffered from severe sepsis, due to ventilator associated pneumonia. All attempts to liberate the patient from the ventilator failed, as the patient could not use the intercostal muscles. A new CSF examination showed normal, serum NMO-IgG, ANA and ACE were negative. The patient underwent tracheostomy and gastrostomy.
A month later, the patient's condition gradually improved, allowing her exemption from the ventilator. A new MRI of the brain was normal (Figure 3A) and most of the intramedullary lesions had vanished (Figure 3B). Her consciousness level improved. Some motion was observed in her arms. Although blindness remained in her right eye, gradual improvement allowed closure of tracheostomy and gastrostomy. However she could not move her lower extremities and had no sense of touch. During the last weeks of hospitalisation she had a persistent bacteriuria by multidrug resistant gram(-) bacteria. She also experienced a right pneumothorax, conservatively treated with success.
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.