An autopsy case of chronic nodular encephalomyelitis simulating neuro-Behçet syndrome. 1986

M Koga, and M Suetsugu, and Y Umeda

A 29-year-old man suffered from neurological symptoms such as urinary disturbance, changes in personality, gait and speech disturbances, amnesia, myoclonus, dysphagia and muscle atrophy for nine years with exacerbations and remissions. A postmortem examination revealed many inflammatory and necrotic foci in the brain and spinal cord, especially in the brainstem and cerebral cortex. Numerous minute scar foci were also scattered in the gray matter, accompanied by some demyelinating lesions in the white matter. The presence of microthrombi and plasma exudation into the vessel wall suggested the importance of vascular factors in the pathogenesis of these CNS lesions. Peripheral neuropathy was also present. The neuropathological features were almost the same as those of neuro-Behçet syndrome, but he lacked the extraneural symptoms of Behçet disease.

UI MeSH Term Description Entries
D008297 Male Males
D009336 Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply.
D001933 Brain Stem The part of the brain that connects the CEREBRAL HEMISPHERES with the SPINAL CORD. It consists of the MESENCEPHALON; PONS; and MEDULLA OBLONGATA. Brainstem,Truncus Cerebri,Brain Stems,Brainstems,Cerebri, Truncus,Cerebrus, Truncus,Truncus Cerebrus
D002531 Cerebellum The part of brain that lies behind the BRAIN STEM in the posterior base of skull (CRANIAL FOSSA, POSTERIOR). It is also known as the "little brain" with convolutions similar to those of CEREBRAL CORTEX, inner white matter, and deep cerebellar nuclei. Its function is to coordinate voluntary movements, maintain balance, and learn motor skills. Cerebella,Corpus Cerebelli,Parencephalon,Cerebellums,Parencephalons
D002536 Cerebral Arteries The arterial blood vessels supplying the CEREBRUM. Arteries, Cerebral,Artery, Cerebral,Cerebral Artery
D002540 Cerebral Cortex The thin layer of GRAY MATTER on the surface of the CEREBRAL HEMISPHERES that develops from the TELENCEPHALON and folds into gyri and sulci. It reaches its highest development in humans and is responsible for intellectual faculties and higher mental functions. Allocortex,Archipallium,Cortex Cerebri,Cortical Plate,Paleocortex,Periallocortex,Allocortices,Archipalliums,Cerebral Cortices,Cortex Cerebrus,Cortex, Cerebral,Cortical Plates,Paleocortices,Periallocortices,Plate, Cortical
D004679 Encephalomyelitis A general term indicating inflammation of the BRAIN and SPINAL CORD, often used to indicate an infectious process, but also applicable to a variety of autoimmune and toxic-metabolic conditions. There is significant overlap regarding the usage of this term and ENCEPHALITIS in the literature. Myeloencephalitis,Encephalomyelitis, Inflammatory,Inflammatory Encephalomyelitis,Myeloencephalitides
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001528 Behcet Syndrome Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well. Behcet Disease,Behcet's Disease,Behcet's Syndrome,Triple-Symptom Complex,Adamantiades-Behcet Disease,Behcet Triple Symptom Complex,Behçet Disease,Old Silk Route Disease,Triple Symptom Complex,Adamantiades Behcet Disease,Adamantiades-Behcet Diseases,Behçet Diseases,Symptom Complex, Triple,Triple Symptom Complices

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