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Paraneoplastic Encephalomyelitis and Subacute Dysautonomia due to an Occult Atypical Carcinoid Tumour of the Lung

Published online by Cambridge University Press:  18 September 2015

M. Veilleux ,
J.P. Bernier  and
J.B. Lamarche
M. Veilleux*
Affiliation:
Department of Neurology, University of Sherbrooke
J.P. Bernier
Affiliation:
Montreal General Hospital and McGill University, and Department of Neurology, University of Sherbrooke
J.B. Lamarche
Affiliation:
Department of Pathology, University of Sherbrooke
*
Division of Neurology, Montreal General Hospital, 1650 Cedar Avenue, Montreal, Quebec, Canada H3G 1A4
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Abstract:

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A case of paraneoplastic encephalomyelitis and subacute pandysautonomia associated with an occult atypical carcinoid tumour of the lung is described. The main clinical features were lethargy, impaired memory, constipation, and orthostatic hypotension. Neurological investigation was unremarkable except for mononuclear pleocytosis and increased protein level in the cerebrospinal fluid (CSF). Tests of autonomic function revealed a low plasma norepinephrine level, a marked drop of blood pressure (BP) to vertical tilt and Valsalva maneuver, and a marked rise of BP to dilute norepinephrine infusion. A few days prior to death, the patient became hypothermic and had repeated episodes of respiratory arrest associated with transient atrioventricular block on the electrocardiogram (ECG). A polysomnography study confirmed a sleep apnea syndrome. Autopsy revealed an atypical carcinoid tumour in one tracheobronchial lymph node, widespread lymphocytic infiltrates and loss of neurones in the cerebral, cerebellar and brainstem gray matter, the spinal cord and roots, and the paravertebral sympathetic ganglia as well as microglial and astrocytic proliferation in the central nervous system.

Résumé:

RÉSUMÉ:

Nous décrivons un cas d'encéphalomyélite paranéoplasique et de pandysautonomie subaiguë associées à une tumeur carcinoïde atypique occulte du poumon. Les manifestations cliniques principales étaient de la létargie, un déficit mnésique, de la constipation et de l'hypotension orthostatique. L'investigation neurologique était sans particularité sauf pour une pléocytose mononucléaire et une augmentation du taux de protéines dans le liquide céphalo-rachidien. Les épreuves de fonction autonome ont montré un taux abaissé de norépinéphrine plasmatique, une chute importante de la tension artérielle (TA) à la bascule verticale et à la manoeuvre de Valsalva, et une élévation marquée de la TA à l'infusion d'une solution de norépinéphrine. Quelques jours avant le décès, le patient a présenté de l'hypothermie et des épisodes répétés d'arrêt respiratoire associés à un bloc auriculo-ventriculaire transitoire à l'électrocardiogramme. Une étude polysomnographique a confirmé la présence d'un syndrome d'apnée du sommeil. L'autopsie a montré une tumeur carcinoïde atypique dans un ganglion lymphatique trachéo-bronchique, des infiltrats lymphocytaires étendus et une perte de neurones dans la substance grise cérébrale, cérébelleuse et du tronc cérébral, la moelle épiniaire et les racines nerveuses, les ganglions sympathiques paravertébraux ainsi qu'une prolifération microgliale et astrocytaire dans le système nerveux central.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 17 , Issue 3 , August 1990 , pp. 324 - 328
Copyright
Copyright © Canadian Neurological Sciences Federation 1990

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