An autopsy case of Schilder's variant of multiple sclerosis (Schilder's disease).
نویسندگان
چکیده
tional cases under the same eponym, but the case reported in 1913 was later identifi ed to be adrenoleukodystrophy, and the 1924 case subacute sclerosing panencephalitis. The diagnostic criteria of ‘true’ Schilder’s disease have been proposed in 1986 [1] . There should be large (at least 3 ! 2 cm) bilateral plaques in the centrum semiovale of the cerebral hemispheres with histological characteristics identical to MS. Here, we report the fi rst case of elderly onset Schilder’s disease confi rmed by auDear Sir, Schilder’s disease or myelinoclastic diffuse sclerosis is a rare form of primary demyelinating disease, which is now considered to be a variant of multiple sclerosis (MS) [1, 2] . In 1912, Schilder described a case of a 14-year-old girl who had signs of mental deterioration associated with an increased intracranial pressure. She died after 19 weeks of her illness, and autopsy revealed large areas of sharply demarcated plaques of demyelination in both hemispheres [3] . Subsequently, Schilder reported two addiReceived: June 13, 2005 Accepted: January 10, 2006 Published online: April 21, 2006
منابع مشابه
Baló's encephalitis periaxialis concentrica.
In 1928, Baló described a law student with an unusual fatal illness marked by aphasia and a right hemiplegia, with later optic neuritis and normal cerebrospinal fluid. At autopsy, he found a disease of the white matter characterised by foci varying in size from a lentil to that of a pigeon's egg and presenting gray softening and, in part, concentricity, where the medullary sheaths were destroye...
متن کاملDevic's neuromyelitis optica and Schilder's myelinoclastic diffuse sclerosis.
An adult patient developed both Devic's neuromyelitis optica and Schilder's myelinoclastic diffuse sclerosis, suggesting that these entities represent rare topographical and aggressive variants within the spectrum of multiple sclerosis.
متن کاملInflammatory myelinoclastic diffuse sclerosis (Schilder's disease): neuroradiologic findings.
Controversy has surrounded the rare clinicopathologic entity of inflammatory myelinoclastic diffuse sclerosis (IMDS) since the seminal description of Schilder in 1912 [1]. Since that time several distinct myelinoclastic and dysmyelinating conditions have been grouped under this eponym. To this newly described demyelinating disease, the author planted the seeds of an enduring controversy by incl...
متن کاملBalo’s concentric lesions with concurrent features of Schilder’s disease in relapsing multiple sclerosis: neuropathological findings
Atypical inflammatory demyelinating syndromes are rare neurological diseases that differ from multiple sclerosis (MS), owing to unusual clinicoradiological and pathological findings, and poor responses to treatment. The distinction between them and the criteria for their diagnoses are poorly defined due to the lack of advanced research studies. Balo's concentric sclerosis (BCS) and Schilder's d...
متن کاملSudanophil leucodystrophy in a pachygyric brain.
Recent classifications of the diffuse cerebral scleroses have recognized the need to subdivide those cases which share in common the feature of sudanophil breakdown products of myelin. In Schilder's disease, as represented by the case he described in 1912, there are large, bilocular, sharply demarcated areas of demyelination in the centrum ovale, often associated with smaller plaques similar to...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- European neurology
دوره 55 2 شماره
صفحات -
تاریخ انتشار 2006