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Anti-Hu associated paraneoplastic sensory neuronopathy with upper motor neurone involvement
  1. M Ogawa1,
  2. M Nishie1,
  3. K Kurahashi1,
  4. M Kaimori2,
  5. K Wakabayashi3
  1. 1Department of Neurology, Aomori Prefectural Central Hospital, Aomori, Japan
  2. 2Department of Clinical Pathology, Aomori Prefectural Central Hospital
  3. 3Department of Neuropathology, Institute of Brain Science, Hirosaki University School of Medicine, Hirosaki, Japan
  1. Correspondence to:
 Dr K Wakabayashi
 Department of Neuropathology, Institute of Brain Science, Hirosaki University School of Medicine, 5 Zaifu-cho, Hirosaki 036-8562, Japan;


Paraneoplastic neurological syndrome is characterised by neuronal degeneration with lymphocytic infiltration in various regions of the central and peripheral nervous systems. Motor neurone symptoms may occur as a remote effect of malignancy, and have been considered because of the involvement of lower motor neurones. A case is reported of an 80 year old woman suffering from paraneoplastic sensory neuronopathy with anti-Hu antibody. Postmortem examination showed adenocarcinoma of the gall bladder and small cell carcinoma of the duodenum. Neuronal loss with lymphocytic infiltration was found in the dorsal root ganglia, brain stem, and cerebellum. Despite the absence of upper motor neurone signs, there was severe loss of Betz cells and degeneration of the bilateral pyramidal tracts. To our knowledge, this is the first demonstration of upper motor neurone involvement in anti-Hu associated paraneoplatic syndrome.

  • CT, computed tomography
  • PEM/PSN, paraneoplastic encephalomyelitis and sensory neuronopathy
  • ALS, amyotrophic lateral sclerosis
  • Betz cell
  • anti-HU antibody
  • necropsy study
  • motor neurone disease
  • paraneoplastic syndrome

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  • Competing interests: none declared