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Letter
Lethal encephalopathy in a patient with isolated nervous system vasculitis
  1. Y Sumitomo1,
  2. M Kunishige1,
  3. N Satake2,
  4. K Shinno3,
  5. M Kawashima4,
  6. T Matsumoto4,
  7. T Mitsui4
  1. 1Department of Medicine and Bioregulatory Sciences, University of Tokushima Graduate School of Medicine, Tokushima, Japan
  2. 2Department of Molecular and Environmental Pathology, University of Tokushima Graduate School of Medicine, Tokushima, Japan
  3. 3Department of Neurosurgery, University of Tokushima Graduate School of Medicine, Tokushima, Japan
  4. 4Department of Medicine and Bioregulatory Sciences, University of Tokushima Graduate School of Medicine, Tokushima, Japan
  1. Correspondence to:
 Dr M Kunishige
 Department of Medicine and Bioregulatory Sciences, University of Tokushima Graduate School of Medicine, 3-18-15 Kuramoto-cho, Tokushima 770-8503, Japan; kuniclin.med.tokushima-u.ac-jp

https://doi.org/10.1136/jnnp.2003.031997

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    Vasculitic neuropathy can occur in patients with connective tissue diseases. On the other hand, non-systemic vasculitic neuropathy has been established as an independent clinical entity, and the risks for systemic spread and death are small.1 In patients with this disorder, vasculitis is limited to the peripheral nervous system (PNS), and histological evaluation is essential for the definitive diagnosis. We encountered a patient with isolated nervous system vasculitis who developed lethal encephalopathy. He had a persistently high titre of anti-GM1 IgG antibody, which is occasionally detected in patients with chronic inflammatory demyelinating polyneuropathy (CIDP).

    Case report

    A 67 year old man had been under treatment for type 2 diabetes for 10 years. In September 1998, he was referred to our hospital because of weight loss and numbness of the lower limbs. He was mentally alert and had exophthalmos. Muscle weakness was prominent in the distal muscles of all four limbs. Sensation was disturbed with a “stocking and glove” distribution. Deep tendon reflexes were diminished in all four extremities.

    Results of laboratory examination indicated diabetes mellitus and hyperthyroidism (haemoglobin A1c 6.8% (normal range 4.3–5.8); thyroid stimulating hormone <0.03 μIU/ml (normal range 0.2–3.2); free triiodothyronine 13.2 pg/ml (normal range 2.9–6.0); free thyroxine 7.65 ng/dl (normal range 0.78–2.10); antithyroglobulin antibody 3200 (normal <100); antithyroid microsomal antibody 26 800 (normal <100); antithyrotropin receptor antibody 16.1% (normal <10); thyroid stimulating antibody 207% (normal <150)). Serological examination for antiganglioside antibodies revealed anti-GM1 IgG, and the titre was 25 900 (normal <800) by enzyme linked immunosorbent assay. No other …

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    Footnotes

    • Competing interests: none declared