Neurologische Komplikationen entzündlicher intestinaler Erkrankungen: Zöliakie, Morbus Whipple, Morbus Crohn und Colitis ulcerosa

Abstract

A number of neurological symptoms have been described as complications of inflammatory bowel disorders such as coeliac disease, Whipple's disease, Crohn's disease and ulcerative colitis. The neurology of coeliac disease includes disorders of the central nervous system encompassing cerebellar ataxia, epilepsy, myoclonus, dementia and multifocal leukoencephalopathy. Peripheral neuropathies, of axonal and demyelinating types, and myopathies have also been reported. The pathomechanisms are more related to immunological-inflammatory processes than to a malresorptive vitamin deficiencies. While CNS symptoms of coeliac disease show a poor response to gluten restriction, peripheral symptoms may respond to elimination of gluten from the diet. There are few reports of a successful corticosteroid treatment in patients showing inflammatory findings in the CSF or bioptic material. Whipple's disease is caused by the gram-positive bacillus Tropheryma whipplei. Patients with a central nervous system manifestation of Whipple's disease may develop a variety of symptoms including dementia, supranuclear gaze palsy, movement disorders, hypothalamic dysfunction and myorhythmia. The CNS-infection is diagnosed by PCR of the CSF. Long-term antibiotic treatment is required, and relapses may occur after withdrawal of antimicrobial therapy. Crohn's disease and ulcerative colitis are complicated by various forms of polyneuropathies, and arterial and venous cerebrovascular diseases. In most cases with neurological complications of inflammatory gastrointestinal diseases an immune mediated inflammatory process is suspected to be the underlying pathomechanism.

Zusammenfassung

Die entzündlichen Darmerkrankungen Zöliakie, Morbus Whipple, Morbus Crohn und Colitis ulcerosa können mit einer Reihe neurologischer Komplikationen einhergehen. Bei der Zöliakie sind Manifestationen am Zentralnervensystem in Form von zerebellärer Ataxie, Epilepsie, Myoklonien, Demenzen und multifokalen Leukenzephalopathien beschrieben. Auch können axonale oder demyelinisierende Polyneuropathien oder selten Myopathien auftreten. Pathogenetisch verantwortlich sind vor allem immunologisch-inflammatorische Mechanismen und nur selten malresorptiv bedingte Vitamindefizite. Während die ZNS-Symptome in der Regel kaum auf eine glutenfreie Kost ansprechen, ist dies bei den peripheren Manifestationen meist der Fall. Daneben sind in Einzelfällen erfolgreiche Kortikosteroidgaben bei bioptisch gesicherter entzündlicher Manifestation beschrieben. Der durch den Erreger Tropheryma whipplei verursachte Morbus Whipple kann im Rahmen einer zerebralen Beteiligung mit Gedächtnis- und Bewusstseinsstörungen, supranukleärer Blickparese, Bewegungsstörungen, hypothalamischen Störungen und Myorhythmien einhergehen. Die Erkrankung lässt sich durch eine PCR im Liquor diagnostizieren und erfordert eine lange antibiotische Therapie, bei deren Absetzen es nicht selten zu Rückfällen kommt. Zu den neurologischen Komplikationen des M. Crohn und der Colitis ulcerosa gehören unterschiedliche Formen von Polyneuropathien sowie arterielle und venöse zerebrovaskuläre Manifestationen. In den meisten Fällen werden die neurologischen Komplikationen entzündlicher Darmerkrankungen durch immunvermittelte Entzündungsprozesse verursacht.

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