Recurrent Polyneuropathy and Neurolymphomatosis

Abstract

polyneuropathy is a rare disorder, especially cases with more than one relapse. Reports of necropsied cases are extremely rare.It is the purpose of this paper to describe the clinical and pathological features of a patient with recurrent polyneuropathy who had many relapses over a period of 31 years.The unusual clinical picture was associated with striking findings at necropsy: there was widespread, but focal, infiltration by lymphoid cells of the peripheral nerves and their ganglia as well as of the leptomeninges and the larger Virchow-Robin spaces of the entire central nervous system (CNS), suggesting a lymphoproliferative process. This combination of clinical and pathological findings has not been previously described in man, but there is considerable resemblance to the disorder known in veterinary medicine as Marek's disease or neurolymphomatosis gallinarum, a polyneuropathy of chickens which recent studies have shown to be of probable viral etiology. Report of a CaseThe patient was in good health until the age of 36 years when, in 1936, in association with an upper-respiratory tract infection, he de¬ veloped marked proximal and distal weakness of his right leg, without numbness, radicular pain, or sphincter disturbances. He recovered incompletely over the next four months and was left with mild atrophy and weakness of that limb.During the next 15 years, on a number of occasions he noted exacerbations of his right leg weakness. These episodes were usually mild but sometimes so severe that he had to be lifted upstairs. He always associated these exacerba¬ tions with infections, usually upper respiratory, or with excessive fatigue. He did not seek medical evaluation for these relapses. He would recover most of his strength in one to two weeks but felt that each exacerbation left him with a slightly greater degree of permanent weakness.In 1951, on the day following a right thoracotomy for resection of a pneumatocele, he de¬ veloped a severe, progressive, flaccid quadriparesis, diffuse fasciculations, virtual areflexia, and mild numbness of his right hand. There was no cranial nerve involvement. He gradually recovered over a period of two months but was left with mild, asymmetrical, proximal, and distal weakness in all extremities.Over the next 16 years, until his death, he experienced one to three exacerbations of his flaccid quadriparesis yearly, usually in associa¬ tion with an upper-respiratory tract infection but sometimes following other types of minor infectious illnesses, surgical procedures, or ex¬ cessive fatigue. There was no definite correla¬ tion between exacerbations and a particular type of medication, such as barbiturates. At times these exacerbations would last only three to six days, but at other times, several weeks were required for recovery, which was often incomplete. Some relapses were accompanied by numbness of his right hand and forearm, but most relapses were entirely motor.In 1963, at the age of 62, he was admitted to the Neurology Service of Stanford University Hospital for evaluation....

B-cell neurolymphomatosis confined to the peripheral nervous system

“…Although the parenchyma of the brain and spinal cord has been spared in some patients with neurolymphomatosis, the meninges have usually been involved [5,6]. This patient was a very rare case of B-cell neurolymphomatosis with lymphomatous infiltration confined to the PNS, although involvement limited to the PNS has been confirmed in a few autopsy cases with histiocytic lymphoma, adult T-cell leukemia, or chronic lymphatic leukemia [7][8][9].…”

Primary Lymphoma of Peripheral Nerve

“…1,15,19,30,37,45,50,53,60 A few cases of neurolymphomatosis have presented with tumor confined largely to the peripheral nervous system. 2,3,6,12,17,34 However, most of these cases have shown concomitant central nervous system (CNS) involvement with either positive cerebrospinal fluid analysis 3,34 or leptomeningeal involvement at autopsy, 2,17 suggesting that peripheral disease results from leptomeningeal spread of tumor cells, 52 a contention supported by the fact that in these patients, peripheral nerve involvement is limited to the proximal portions of the nerves.…”

Guillain-Barré syndrome and head trauma: case report

“…There are reports that describe the reativation of the GBS in patients who had a previous history of the disease once they were submitted to surgical procedures 4 .…”