An autopsy case of acute multiple sclerosis (Marburg's type) during pregnancy.
We report a case of a 9-month pregnant woman who presented acute psychiatric and neurological symptoms with extensive involvement of the white matter on MRI and no oligoclonal bands on CSF examination. Despite high doses of intravenous steroids, plasmapheresis and immunosuppressive drugs, a fatal outcome (coma) was noted 8 months later. Neuropathological examination confirmed the diagnosis of Marburg's type of multiple sclerosis showing sharp-edged lesions of demyelination, giant astrocytes, numerous macrophages and little perivascular inflammation. We discuss the definition and limits of the Marburg entity with reference to acute disseminated encephalomyelitis, impact of pregnancy, unusual MRI features, neuropathology and treatment.
Letournel
F
F
UF de Neurobiologie et Neuropathologie, Département de Pathologie Cellulaire et Tissulaire, CHU, 4 rue Larrey, 49033 Angers, France.
Cassereau
J
J
Scherer-Gagou
C
C
Bernard
I
I
Mercat
A
A
Gray
F
F
Tanguy
J Y
JY
Richard-Crémieux
I
I
Jeanfaivre
Th
T
Barthelaix
A
A
Dubas
F
F
D000208:Acute Disease; D000328:Adult; D001921:Brain / Q000473:pathology*; D003937:Diagnosis, Differential; D004673:Encephalomyelitis, Acute Disseminated / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D008279:Magnetic Resonance Imaging; D009103:Multiple Sclerosis / Q000188:drug therapy / Q000473:pathology*; D010956:Plasmapheresis; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy / Q000473:pathology*; D013256:Steroids / Q000627:therapeutic use
D013256:Steroids