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No. 74 (PP 7)
Neuropathological findings in a case of amyotrophic lateral sclerosis resembling
CIDP
Masaru Yoshioka (1), Hidehiko Konno (1), Toshiaki Takahashi (1), Hiroyasu Tanaka (1), Hiroshi
Onodera (1), Maki Tateyama (2)
(1) Department of Neurology, National Hospital Organization Nishitaga Hospital
(2) Department of Neurology, Tohoku University School of Medicine, Japan
Aim: We present a case fulfilling diagnostic criteria of chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP) with a progressive course refractory to immunosuppressive therapy, and
showing autopsy findings indicative of amyotrophic lateral sclerosis.
Methods: A clinicopathological case report.
Results: A patient, 62 year-old male, developed motor weakness, muscle atrophy, and sensory deficits in
both upper and lower limbs in a progressive course. On examinations, cerebrospinal fluid showed
protein-cellular dissociation, neurophysiological studies revealed reduced motor and sensory nerve
conduction velocities with prolonged distal latencies in multiple nerves. Biopsy of sural nerve showed
segmental demyelination in teased fibers, fulfilling diagnostic criteria of definite CIDP. Systemic motor
weakness and muscle atrophy progressed after repeated intravenous immunoglobulin infusions and
steroid pulse therapies. Patient died of respiratory failure after the disease duration of 3 years and 5
months. Autopsy revealed diffuse loss of myelinated fibers in anterior and posterior roots. Cellular loss
of posterior root ganglia was observed as well. Sciatic nerve showed demyelination and perineurial
edema. Anterior horn cells were massively reduced and pallor of lateral columns was found along the
whole levels of spinal cord. Iliopsoas muscle and diaphragm exhibited marked neurogenic atrophy.
Some brainstem nuclei showed neuronal loss.
Conclusions: The present case suggests that an association of amyotrophic lateral sclerosis may be a
factor for
therapeutic refractoriness in some patients with polyneuropathy resembling CIDP. Causal relationship
between demyelinating neuropathy and loss of anterior horn cells is uncertain.
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