NEUROPATHY IN ANGIOIMMUNOBLASTIC LYMPHADENOPATHY

  • 1 January 1981
    • journal article
    • research article
    • Vol. 137  (8-9) , 503-515
Abstract
Four [human] cases of angioimmunoblastic lymphadenopathy (AIL) associated to peripheral neuropathy are described. The neuropathy was mixed, sensory and motor, more or less extensive and always asymetrical. In 2 cases, the clinical symptomatology and the clinical course were very peculiar, characterized by sensory disorders of a precise topography, circumscribed and sometimes suspended and by a relapsing and remitting course. In the 3rd case, the neurological signs were acute and rapidly extensive with mandatory respiratory assistance. In this case, death occured after a few weeks; the exact diagnosis was only attained at post-mortem examination. In the 4th case the neuropathy was very painful but the course was slow. In all 4 cases marked and extensive pain was present prior to the neurological disorders. Electrophysiological abnormalities were a constant feature with a marked slowing down of nerve conduction velocity. CSF was normal at the beginning in 1 case but was otherwise markedly pathological with an increased number of cells due to a large number of lymphocytes ranging from 6-40 cells while protein ranged from 60-160 mg/100 ml. Nerve and muscle biopsies were nonspecific, i.e., neurogenous muscular atrophy and demyelination, except in case 4 where specific angioimmunoblastic lymphadenopathy infiltrates were present both in nerve and muscle. In cases 1 and 3 a nonspecific lymphohistiocytic infiltrate was present in spinal roots and meninges. Corticotherapy was effectively used in 2 cases. These data are compared with a review of the literature. Since 1976, 7 cases of AIL associated to peripheral neuropathy have been reported. Clinical, electrophysiological and biological features are similar. Only 1 case underwent a post-mortem examination of the CNS; a nonspecific lymphocytic infiltration in the spinal roots and meninges was mentioned. The role of the dysproteinemia associated with the AIL in the occurrence of the neuropathies is discussed. It remains a possible factor only. The role of a massive specific localization of the pathological process is well established in only 1 case (case 4). Such localizations in lymphomas are not unusual. In 2 of these cases as well as in 2 from the literature the pathological findings are nonspecific, mild lymphoplasmocytic infiltrates of spinal roots and meninges. Those lesions are similar to neuropathies associated to nonmetastasizing neoplasms or malignant hemopathies.

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