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Multiple sclerosis-ichthyosis-factor VIII deficiency syndrome

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Disease definition

Multiple sclerosis-ichthyosis-factor VIII deficiency syndrome is characterized by the association of multiple sclerosis with lamellar ichthyosis and hematological anomalies (beta thalassemia minor and a quantitative deficit of factor VIII-von Willebrand complex). Other clinical manifestations may include eye involvement (optic atrophy, diplopia), neuromuscular involvement (ataxia, pyramidal syndrome, gait disturbance) and sensory disorder. There have been no further descriptions in the literature since 1992.

ORPHA:3151

Classification level: Disorder

Source: PubMed ID 8336172

Prevalence: <1 / 1 000 000

Inheritance: Unknown

Age of onset: Childhood

ICD-10: G37.8

ICD-11: 8A4Y

UMLS: C4518551

A summary on this disease is available in Français, Español, Deutsch, Italiano, Nederlands
Detailed information

Logo ERN: produced/endorsed by ERN(s) Logo FSMR: produced/endorsed by FSMR(s)

Guidelines
Emergency guidelines
Français (2018.pdf) - Orphanet Urgences
Clinical practice guidelines
Disease review articles
Clinical genetics review
English (2013.pdf) - Eur J Hum Genet
The documents contained in this website are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.