Diffuse Cerebral Sclerosis of Schilder

Schilders diffusa cerebrala skleros

Svensk definition

Ett sällsynt tillstånd av myelinbortfall i det centrala nervsystemet som ffa drabbar barn och unga människor. Patologiska kännetecken är bl a en stor och tydligt avgränsad och asymmetrisk koncentration av myelinnedbrytning, som kan omfatta en hel lob eller hjärnhalva. Det kliniska förloppet är oftast progressivt och inkluderar demens, kortikal blindhet och dövhet, spastisk hemiplegi och pseudobulbärpares. Balos koncentriska skleros skiljs patologiskt från Schilders diffusa cerebralskleros genom förekomsten av koncentriska ringar av nedbrutet respektive bevarat myelin. Alpers syndrom avser en icke enhetlig grupp sjukdomstillstånd med tecken på fortskridande cerebralt förfall och leversjukdom.

Engelsk definition

A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73)

Svenska synonymer

Alpers syndrom Balos koncentrisk skleros Encephalitis periaxialis Schilders sjukdom Periaxial encefalit

Engelska synonymer

Encephalitis Periaxialis Encephalitis Periaxialis Concentrica Sudanophilic Cerebral Sclerosis Myelinoclastic Diffuse Sclerosis Diffuse Scleroses, Myelinoclastic Diffuse Sclerosis, Myelinoclastic Myelinoclastic Diffuse Scleroses Scleroses, Myelinoclastic Diffuse Sclerosis, Myelinoclastic Diffuse Poliodystrophia Cerebri Schilder Disease Disease, Schilder Schilder's Disease Disease, Schilder's Schilders Disease Cerebral Sclerosis, Diffuse Diffuse Cerebral Scleroses Diffuse Cerebral Sclerosis Sclerosis, Diffuse Cerebral Encephalitis Periaxialis Diffusa Balo Concentric Sclerosis Concentric Sclerosis, Balo Balo's Concentric Sclerosis Concentric Sclerosis, Balo's Scleroses, Balo's Concentric Alpers Syndrome Syndrome, Alpers Alpers Disease Alpers Progressive Infantile Poliodystrophy Progressive Sclerosing Poliodystrophy Progressive Sclerosing Poliodystrophies Alpers' Syndrome Alper Syndrome Alper's Syndrome Alpers-Huttenlocher Syndrome Alpers Huttenlocher Syndrome Syndrome, Alpers-Huttenlocher Neuronal Degeneration Of Childhood With Liver Disease, Progressive Progressive Neuronal Degeneration of Childhood with Liver Disease Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis Alpers' Disease Alper Disease Alper's Disease Disease, Alpers'