Isolated severe vasculitic neuropathy revealing sarcoidosis

• Published in: Lancet neurology 2008-08, Vol.7 (8), p.756-760
• Main Authors: Souayah, Nizar, MD, Chodos, Ari, MD, Krivitskaya, Natalia, MD, Efthimiou, Petros, MD, Lambert, W Clark, MD, Sharer, Leroy R, MD
• Format: Article
• Language: English
• Subjects: Biopsy; Humans; Immunology; Laboratories; Male; Medical treatment; Middle Aged; Muscle, Skeletal - pathology; Neural Conduction - physiology; Neurology; Patients; Peripheral Nervous System Diseases - diagnosis; Peripheral Nervous System Diseases - physiopathology; Sarcoidosis; Sarcoidosis - diagnosis; Sarcoidosis - physiopathology; Studies; Tomography, X-Ray Computed; Vascular Diseases - diagnosis; Vascular Diseases - physiopathology
• ISSN: 1474-4422; 1474-4465
• DOI: 10.1016/S1474-4422(08)70166-2

A detailed laboratory and immunological examination was normal, including complete blood count, metabolic panel, urinalysis, serum protein electrophoresis, cryoglobulins, autoimmune serologies, antibodies for HIV, hepatitis B and C, Lyme disease, antineutrophil cytoplasm antibody, and complement concentrations. Peripheral neuropathy is a rare neurological complication of sarcoidosis, occurring in 15-18% of cases.\n16 Other therapies, such as cyclosporine and plasma exchange, can be used in refractory patients.16 The likelihood of non-systemic vasculitic neuropathy evolving into a systemic vasculitis varies from 6% to 34% over several years of follow-up.18,20,21 The risk of relapse varies from 34% to 46%, overlapping with that of systemic vasculitis.16 In our patient, the presence of non-caseating granulomas on mediastinal lymph-node biopsy, the dramatic response to immunosuppressive therapy, and the absence of relapse after 15 months' follow-up favour the diagnosis of sarcoid vasculitic neuropathy.  Spontaneous Motor unit action potentials Recruitment pattern[dagger]  Insertional activity Fibrillation* Positive sharp waves* Fasciculations Other Amplitude Duration Polyphasic Fast firing  Right side Tibialis anterior Increased 3+ 3+ 0 0 Increased Increased Increased Increased Low mixed Vastus lateralis Increased 0 0 0 0 Increased Increased Normal Increased Full Abductor digiti minimi Increased 3+ 3+ 0 0 Increased Increased Increased Increased Low mixed First dorsal interosseous Increased 4+ 4+ 0 0 0 0 0 0 0 Flexor carpi ulnaris Increased 0 1+ 0 0 Increased Increased Increased Increased Low mixed Deltoid Normal 0 0 0 0 Normal Normal Normal 0 Full Biceps Normal 0 0 0 0 Normal Normal Normal 0 Full Left side Tibialis anterior Increased 3+ 3+ 0 0 Increased Increased Increased Increased Low mixed Gastrocnemius (median) Increased 2+ 2+ 0 0 Increased Increased Increased Increased Low mixed Abductor digiti minimi Increased 2+ 3+ 0 0 Increased Increased Increased Increased Low mixed Flexor carpi ulnaris Increased 0 1+ 0 0 Increased Increased Increased Increased Low mixed Deltoid Increased 0 0 0 0 Normal Normal Normal 0 Full Table 2 Needle electromyogram examination Spontaneous effort was normal for all muscle groups.