Neurosarkoidose: Diagnostik und Therapie

Abstract

Sarcoidosis is an inflammatory systemic disease with a prevalence of 20 to 50 of 100,000 inhabitants in West-Germany. Caused by an abnormal immune response to a hypothetic inhalative antigen, a granulomatous inflammation in mediastinal lymph nodes occurs, which frequently extends to the lung and sometimes to other tissues. The nervous system is clinically involved in 5% of the cases. The most frequent neurologic symptom is solitary or combined cranial nerve involvement. Cerebral manifestations are nodular or diffuse granulomatous infiltration of the brain and lymphocytic inflammation of the basal meninges. The rarer myelopathies and peripheral neuropathies often cause major neurological deficits. Muscular involvement, which is present in about half of the cases of sarcoidosis, normally causes clinically silent lesions. Chronic sarcoid myopathy or acute polymyositis are rare. Elements of the diagnostic procedure are X-ray of the lung, measurement of serum Angiotensin-Converting Enzyme (ACE)- and serum Lysozyme (LZM)-levels, Bronchoalveolar lavage, Gallium-67-scanning and Kveim-test. The diagnostic value of these investigations and the typical findings in CSF examination, computed tomography, nuclear magnetic resonance, myelography and electrophysiological investigation are presented. Suspected diagnosis of sarcoidosis should be confirmed by biopsy of non-caseating granulomatous lesions in involved tissues. An initially high dosage corticosteroid therapy for many months to several years is often effective. If this is not helpful in cerebral sarcoidosis whole brain irradiation with 1500 to 3000 rd administered in 10 single doses is indicated. Peripheral neuropathy refractory to therapy with orally administered cortisone should be treated with parenteral ultrahigh steroid therapy.

Einseitige Optikusneuropathie als Zeichen einer Neurosarkoidose

“…Gezielte Organbiopsien (Konjunktiven, Lymphknoten, granulomatose Herde) sind beweisend, aber oft nicht durchfuhrbar. Das hochste Mass an Spezifitat and Sensitivitat zusammen besitzt der Kveim-Test, der bei Unklarheiten oder fehlender Biopsiemoglichkeit uber spezialisierte Zentren zu erhalten ist [13].…”

“…Schlegel [13] empfiehlt 80-120mg Prednisolon taglich uber 2 Monate mit langsamer Dosisreduktion bis zu 30 mg taglich nach 18-24 Monaten, da Ruckfalle nach zu fruhem Absetzen haufig sind. Die Mindest-Therapiedauer sollte 4-6 Monate betragen.…”

“…Auch das Nervensystem ist klinisch in lediglich 5-1070 der Sarkoidosefalle mitbeteiligt [13] Eine neuerliche stationare Behandlung mit parenteralen Steroidgaben erbrachte nun keine wesentliche Sehverbesserung mehr, der Visus stabilisierte sich bei 0,2p.…”

Neurosarcoidosis without systemic sarcoidosis

“…The major pathological features are aseptic granulomatous basal meningitis or focal lesions due to granulomas; common neurological symptoms are facial palsy, other cranial neuropathies, and involvement of central nervous system (CNS) parenchyma causing hypothalamicpituitary dysfunction, encephalopathy, seizures, and lesions of the corticospinal tract [5,10,15,19,22,26]. Cerebral sarcoidosis without systemic manifestation is rare [15,22,26] and difficult to diagnose.…”

Correlation of magnetic resonance imaging findings and histopathology of lesion distribution of spinal cord sarcoidosis atpost‐mortem

“…Sarcoidosis is a systemic non‐caseating granulomatous disease that most commonly affects the lungs, skin, lymph nodes, bones, eyes, heart, kidneys, and parotid glands. Nervous system involvement, so‐called neurosarcoidosis, is found in 5–10% of patients [20,23]. Neurosarcoidosis most commonly involves the brain and peripheral nerves and often occurs in the early stages of the disease [5,10].…”