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Byline: Romain Sonneville (1), Sophie Demeret (2), Isabelle Klein (3), Lila Bouadma (1), Bruno Mourvillier (1), Juliette Audibert (4), Stephane Legriel (5), Francis Bolgert (2), Bernard Regnier (1), Michel Wolff (1) Keywords: Encephalomyelitis; Demyelination; Post-infectious; Adults; Vaccination Abstract: Objective Because acute disseminated encephalomyelitis (ADEM) is a rare disease in adults admitted to the intensive care unit (ICU), we describe its characteristics and patient outcomes. Design and setting A retrospective (2000--2006), observational, multicenter study was conducted in seven medical ICUs. Clinical, biological and neuroimaging features of patients diagnosed with ADEM were evaluated. Functional prognosis was graded using the modified Rankin (mR) scale. Interventions None. Measurements and results At ICU admission, the 20 patients' median (25th--75th percentile) Glasgow coma score (GCS) was 7 (4--13), temperature 39 (38--39)a-degC. Six (30%) patients had seizures, 17 (85%) had a motor deficit and 14 (70%) required mechanical ventilation. Fifteen (75%) patients had cerebrospinal fluid pleocytocis. All patients had white-matter lesions on their magnetic resonance images. All patients received high-dose steroids. Five (25%) patients died. Fourteen (70%) patients were able to walk without assistance (mRa-a$?a-3) at follow-up [7 (3--9) months]. Compared to the latter, patients who died or were severely disabled at the follow-up evaluation [6 (30%) patients, mRa->a-3] had significantly lower GCS (4 (3--4) vs. 12 (7--13), pa-=a-0.002) and more frequent seizures [4 (67%) vs. 2 (14%), pa-=a-0.02] at admission. Conclusions Unlike previous reports, our results showed that ADEM requiring ICU admission is a severe disease causing high mortality, and 35% of the patients had persistent functional sequelae. Intensivists should be aware of ADEM's clinical features to initiate appropriate immunomodulating therapy. Author Affiliation: (1) Service de Reanimation Medicale et des Maladies Infectieuses, Hopital Bichat--Claude Bernard, AP--HP, Universite Paris 7, 46, rue Henri Huchard, 75877, ParisCedex 18, France (2) Service de Neurologie I, Groupe Hospitalier Pitie--Salpetriere, AP--HP, Universite Paris 6, 47, boulevard de l'Hopital, 75651, Paris Cedex 13, France (3) Service de Radiologie, Hopital Bichat--Claude Bernard, AP--HP, Universite Paris 7, 46, rue Henri Huchard, 75877, ParisCedex 18, France (4) Service de Reanimation Medicale, Hopital Europeen Georges Pompidou, AP--HP, Universite Paris 5, 20, rue Leblanc, 75908, Paris Cedex 15, France (5) Service de Reanimation Medicale, Hopital Saint-Louis, AP--HP, Universite Paris 7, 1, avenue Claude Vellefaux, 75475, Paris Cedex 10, France Article History: Registration Date: 16/10/2007 Received Date: 06/06/2007 Accepted Date: 14/10/2007 Online Date: 09/11/2007 Article note: Electronic supplementary material The online version of this article (doi: 10.1007/s00134-007-0926-2) contains supplementary material, which is available to authorized users. more...

Byline: Brigitte Wildemann (1), Olav Jansen (2), Juergen Haas (1), Maria-Elisabeth Vogt-Schaden (1), Brigitte Storch-Hagenlocher (1) Keywords: Key words Demyelinating disease; Central nervous system lymphoma; Polymerase chain reaction; Complementarity determining region 3IgH; Gene scanning Abstract: Polymerase chain reaction (PCR) based automated high-resolution fragment analysis of rearranged immunoglobulin heavy-chain genes is a highly sensitive means for identifying clonal B-cell responses. We used this technique to distinguish polyclonal inflammatory from monoclonal neoplastic B-cell populations in the cerebrospinal fluid (CSF) of three patients with acute demyelinating disorders of the central nervous system whose clinical, magnetic resonance imaging (MRI) and CSF features did not permit unequivocal exclusion of primary central nervous system lymphoma (pC-NSL). This approach is highly suitable for detecting CNS inflammation particularly when lymphomatous involvement cannot be ruled out by noninvasive diagnostic procedures alone. Author Affiliation: (1) Department of Neurology, University of Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany, Tel.: +49-62 21-56 75 04, Fax: +49-62 21-56 54 61, e-mail: brigitte_wildemann@med.uni-heidelberg.de, DE (2) Department of Neuroradiology, University of Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany, DE Article note: Received: 31 May 2000 / Received in revised form: 14 August 2000 / Accepted: 22 August 2000 more...

A 15-year-old girl was admitted to a hospital when she developed slurred speech, partial paralysis and difficulty speaking. A CT scan revealed a single abnormality, which her doctors suspected was a tumor. This would be the most likely cause of this kind of abnormality in a young person. However, a brain biopsy showed that she had a demyelinating disease similar to multiple sclerosis. She was treated with corticosteroids and cyclophosphamide. more...

A 23-year-old healthy man developed tingling and numbness of his feet and hands, leg weakness, poor balance, and an arm tremor over a six-month period. Extensive testing ruled out infectious, toxic, drug-related, or other causes, and documented a pattern of symptoms suggestive of a neurologic disorder. He was diagnosed with chronic inflammatory demyelinating polyradiculopathy, a degenerative nerve condition. He responded to antiinflammatory medication, although plasma exchange and immune globulin can be effective when an autoimmune process is involved. more...

Byline: Karl-Olof Lovblad (1), Gianpaolo Ramelli (2), Luca Remonda (1), Arto C. Nirkko (3), Christoph Ozdoba (1), Gerhard Schroth (1) Abstract: Vitamin B.sub.12 deficiency is known to be associated with signs of demyelination, usually in the spinal cord. Lack of vitamin B.sub.12 in the maternal diet during pregnancy has been shown to cause severe retardation of myelination in the nervous system. We report the case of a 14 1/2-month-old child of strictly vegetarian parents who presented with severe psychomotor retardation. This severely hypotonic child had anemia due to insufficient maternal intake of vitamin B.sub.12 with associated megaloblastic anemia. MRI of the brain revealed severe brain atrophy with signs of retarded myelination, the frontal and temporal lobes being most severely affected. It was concluded that this myelination retardation was due to insufficient intake of vitamin B.sub.12 and vitamin B.sub.12 therapy was instituted. The patient responded well with improvement of clinical and imaging abnormalities. We stress the importance of MRI in the diagnosis and follow-up of patients with suspected diseases of myelination. Author Affiliation: (1) Department of Neuroradiology, IDR, Inselspital, University of Bern, Freiburgstrasse, CH-3010, Bern, Switzerland (2) Department of Pediatrics, Inselspital, University of Bern, Switzerland (3) Department of Neurology, Inselspital, University of Bern, Switzerland Article History: Registration Date: 28/03/2009 Received Date: 19/02/1996 Accepted Date: 26/03/1996 more...

A case history is reported of a 28-year-old woman who was diagnosed with demyelinating disease. Over a one-month period her symptoms progressed from a toothache to severe headaches and weight loss. She experienced a major personality change, intellectual deficits and lost her ability to speak. A CT scan and magnetic resonance imaging (MRI) revealed lesions in the white matter of her brain. Possible diagnoses were infection, cancer, inflammatory vascular disease, and central nervous system disease. Her history, symptoms, imaging studies, and laboratory tests were compatible with either an unusual manifestation of multiple sclerosis or acute disseminated encephalomyelitis. The latter may be a type of autoimmune disease. Acute disseminated encephalomyelitis was chosen because the consequences of misdiagnosis were less serious than an initial misdiagnosis of multiple sclerosis. Administration of immunosuppressants relieved her symptoms. Eight months later an MRI scan showed marked shrinkage of the brain lesions. more...

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Chronic inflammatory demyelinating polyneuropathy (CIDP) cannot generally be diagnosed on the basis on one or two key observations. The disorder, in which an inflammatory process slowly destroys the myelin sheaths (an important covering material of nerve cells) of peripheral nerves, must be diagnosed on the basis of a constellation of different clinical signs, laboratory values, and physiological measurements. A subcommittee of the American Academy of Neurology AIDS Task Force has compiled a set of criteria for the diagnosis of CIDP. For the diagnosis of CIDP, a patient must have progressive or relapsing dysfunction involving at least two limbs and lasting more than two months. The dysfunction may be motor or sensory, but is usually both. The patient must have reduced or absent reflexes. If the patient has unequivocal sphincter problems, the condition is not CIDP and another diagnosis must be considered. A patient with CIDP will always have nerve conduction abnormalities, which may be demonstrated by neurophysiologic studies. These problems may include a reduction in nerve conduction velocity or signs of a nerve block. To qualify for a diagnosis of CIDP, a patient's nerve biopsy must show signs of demyelination or remyelination. The inflammation of blood vessels, the presence of the protein complex amyloid, or swollen nerve axons are among the pathological indications that some other disease process besides CIDP is at work. If cerebrospinal fluid is drawn from the patient, the cell count must be less than 10 cells per cubic millimeter for the diagnosis of CIDP to be considered definite. If the patient is HIV-positive, then 50 cells per cubic millimeter is the limit. By definition, CIDP which occurs in the absence of some other disease process is called idiopathic CIDP. However, CIDP may occur as a part of a number of different syndromes including HIV infection, systemic lupus erythematosus, macroglobulinemia and gammopathies, Castleman disease, diabetes. Chronic inflammatory demyelinating polyneuropathy may also occur along with syndromes of demyelination in the central nervous system. (Consumer Summary produced by Reliance Medical Information, Inc.) more...

Magnetisation transfer ratio (MTR) measurement seems promising as a putative marker of demyelination in the CNS. Small MTR reductions may also occur as a result of axonal loss, oedema, or [...]