1/33. T cell-mediated paraneoplastic ganglionitis--an autopsy case. A 57-year-old woman presented with subacute sensory, ataxic neuronopathy. Clinical investigation revealed a right-sided non-small-cell lung cancer. serum investigation for specific antineuronal antibodies was negative. histology showed T lymphocytic infiltrates in dorsal root ganglia. The observed histological pattern is similar to that described in antibody-positive cases. Thus, these findings suggest similar pathways in specific antineuronal antibody-negative and -positive cases of paraneoplastic subacute sensory neuronopathy. ( info) |
2/33. Morphometric evaluation of paraneoplastic neuropathies associated with carcinomas, lymphomas, and dysproteinemias. Paraneoplastic peripheral neuropathies are caused by indirect effects of carcinomas, mainly small cell bronchogenic carcinomas, lymphoproliferative disorders (lymphomas, myelomas, polycythemia vera), and dysproteinemias (benign monoclonal paraproteinemia, Waldenstrom's macroglobulinemia) including cryoglobulinemias. Those associated with carcinomas are usually considered as severe, those associated with benign gammopathies (monoclonal gammopathies of unknown significance, MGUS) as mild, and those with cryoglobulinemias as of variable severity. In a larger series of 104 autopsy and biopsy cases, we noted a wide range of severity concerning various morphometric parameters of peripheral nerve fibers by evaluating sural nerves. There were no apparent morphometric differences between the groups of disorders. The most valuable parameter of optic-electronic evaluation and comparison turned out to be the myelin area expressed as a percentage of the endoneurial area because this measure comprises the relative number, size, and myelin thickness of the myelinated nerve fibers. In the 104 cases of the three disease groups, most of the cases (38 cases; 36.5%) showed a moderate reduction of the myelin sheath area per endoneurial area of sural nerves. This was followed by 34 cases (32.7%) with severe and very severe reduction. Twenty-nine cases (27.9%) presented with mild reduction. It is concluded that the severity of the neuropathy depends largely on the stage of the disease and the time of progression rather than on the type of the underlying disorder. ( info) |
3/33. chorea resulting from paraneoplastic striatal encephalitis. A 73 year old man presented with progressive choreic movement and dementia. An antineuronal antibody that recognised a 68 kDa band on a western blot was found in the patient's serum; this antibody immunolabelled neuronal somata in rat brain. Postmortem examination showed a small cell lung cancer and severe neuronal loss with lymphocytic infiltration in the striatum that was more severe in the caudate head. This is thought to be the first pathologically proved case of paraneoplastic chorea with striatal encephalitis. ( info) |
4/33. Successful immunosuppressant therapy of severe progressive cerebellar degeneration and sensory neuropathy: a case report. A 56 year old woman had a 19 month history of a severe subacute progressive cerebellar degeneration, peripheral sensory neuropathy, and urinary incontinence. She was confined to a wheelchair, needed assistance with eating, and her speech was almost unintelligible. No underlying cancer was found despite repeated investigations, and no autoantibodies were demonstrated. She received a 3-month course of intensive immunosuppressant therapy with intravenous immunoglobulin 400 mg/kg per day for 5 days every month, oral cyclophosphamide 50 mg twice or three times a day to maintain the total lymphocyte count between 500 and 750/mm(3), and prednisone 60 mg per day. She experienced dramatic subjective and objective improvement. The dysarthria and the upper extremity dysmetria disappeared, and she regained the ability to write and cook. The lower extremity ataxia improved and she became able to walk with a cane. urinary incontinence disappeared. A trial of intensive immunosuppressant treatment is worth considering in a patient with a clinical syndrome resembling paraneoplastic disorders, even if an underlying neoplasm and autoantibodies are not demonstrated. ( info) |
| A patient with painful peripheral neuropathy is presented, whose symptoms were thought to result from an infection with borrelia burgdorferi sensu lato. Investigations of the cerebrospinal fluid for signs of inflammation and borrelial antibodies were negative, and the patient did not benefit from repeated antibiotic treatment. Electrophysiologic studies and sural nerve biopsy showed axonal neuropathy consistent with a paraneoplastic syndrome. Further workup revealed mediastinal Hodgkin's disease (HD; nodular sclerosing subtype) Ann Arbor stage II and non-small cell cancer of the lung (stage T1N0M0). Surgical resection of the lung cancer and combined chemo- and radiotherapy for HD resulted in complete remission of both malignancies. While the preexisting neurologic symptoms persisted during treatment, neurography showed some improvement of the distal nerves. During radiation therapy the patient developed transient left-sided brachial plexopathy. This case illustrates that the diagnosis of borreliosis in patients with isolated painful peripheral neuritis cannot be based solely upon positive IgG titers and supports the requirement for a thorough workup for an underlying--potentially curable--disease. In addition, singular pulmonary lesions in the setting of HD should be suspected to have a separate cause. ( info) |
6/33. Improvement of anti-Hu-associated paraneoplastic sensory neuropathy after chemoradiotherapy in a small cell lung cancer patient. A 66-year-old man developed progressive painful dysesthesia in his hands and feet over 3 months. His vibration sense was impaired and sensory nerve action potentials of the limbs were not evoked. biopsy of the peroneal nerve revealed sensory neuropathy. Positive anti-Hu antibody facilitated delineation of a right hilar mass and a metastatic lymph node in thoracic CT scan. He was diagnosed as small cell lung cancer associated with paraneoplastic sensory neuropathy. A complete response was achieved through chemotherapy (carboplatin and etoposide) and subsequent radiation therapy. Notably, his neurological conditions, although not changed during the hospitalization, gradually improved afterwards. ( info) |
7/33. A clinicopathological study of a patient with anti-Hu-associated paraneoplastic sensory neuronopathy with multiple cranial nerve palsies. Only a few cases of paraneoplastic neurologic syndrome with multiple cranial palsies have been reported. This is the case report of a patient with small-cell lung cancer and a high titer of anti-Hu antibodies who developed a tonic left pupil and multiple cranial nerve palsies, including palsies of the left fifth through tenth nerves and both twelfth nerves, as in Garcin syndrome showing at least more than seven ipsilateral cranial nerve palsies, in the course of paraneoplastic sensory neuronopathy (PSN). Pathologic examination revealed no metastasis or direct invasion of malignancy with gliosis and perivascular inflammation throughout the brainstem, indicating paraneoplastic encephalomyelitis (PEM). The numbers of EBM11 cells (probably reactive microglia), CD8 cells, and CD4 cells increased. Intracellular adhesion molecule-1 and lymphocyte function associated molecule-1 were expressed intensely on the endothelia of microvessels and were found to have infiltrated mononuclear cells around microvessels in the brainstem. Multiple cranial nerve palsies and their effects including the tonic pupil are likely due to the paraneoplastic effect of the primary systemic malignancy. ( info) |
8/33. Peripheral paraneoplastic neuropathy, an uncommon clinical onset of sigmoid cancer. Case report and review of the literature. A case of a 76-year-old man presenting with weakness of the lower legs and bilateral steppage gait is described. Neurological examination revealed a sensorimotor neuropathy with axonopathy and myelinic aspects. At the time of the diagnostic workup an episode of rectal bleeding occurred. colonoscopy demonstrated an exophytic cancer of the sigmoid colon at 40 cm from the anal verge. At surgery the tumor adhered to the ileum, so a left hemicolectomy and ileo-ileal resection were performed. Tumor stage was Dukes' B, Jass III, Astler-Coller B2, T3N0M0. The patient underwent postoperative chemotherapy and was followed for the past three years. At present he is free of disease and the neuropathy has completely regressed without any dedicated therapy. As reported in the literature the appearance of a paraneoplastic neurological syndrome (PNS) may be the first sign of a malignancy that is occult at the time of clinical presentation. The most widely supported theory about its etiology is that of an autoimmune origin. The paraneoplastic neurological syndrome is considered to be at a point of intersection between tumor immunology, autoimmune neurological disease, and basic neurobiology. Previous experience has resulted in a pathogenic model and in a definition of a group of autoantibodies related to the disease. Small cell lung cancer (SCLC) is the neoplasm most frequently associated with PNS; other malignancies include lymphomas and various hematological malignancies. Some authors reported also that the percentage of patients with a high titer of neuronal autoantibodies is small and several of the autoantibodies are present at low levels without any accompanying clinical manifestation. In a clinical retrospective study of the Mayo Clinic Group 115,081 patients were examined over the period 1984-1993 and only 58 patients (0.05%) could be defined as being affected by a paraneoplastic neurological syndrome. Only five of these patients had colon tumors. The number of patients is so small and so widely scattered among publications that no statistical analysis is possible. Probably the only possibility for early identification of such a syndrome is a high degree of suspicion. In fact, these patients are usually first admitted and studied in a neurological unit, and the diagnosis of a tumor-associated disease is a delayed event. ( info) |
9/33. Life-threatening polyneuropathy heralding renal cell carcinoma. OBJECTIVE: To report the occurrence of life-threatening polyneuropathy in association with renal cell carcinoma. methods: Case report and review of the medical literature. RESULTS: A 65-year-old man developed a fulminant, life-threatening sensory motor polyneuropathy several months before renal cell carcinoma was identified. Dramatic symptomatic improvement ensued after treatment with intravenous immunoglobulin, although the patient succumbed to complications of the tumor years later. CONCLUSIONS: Chronic sensory motor polyneuropathy is a known paraneoplastic syndrome associated with renal cell carcinoma. This report emphasizes that in rare conditions, the polyneuropathy could take an acute demyelinating form, which is important for clinicians to recognize. ( info) |
10/33. A case of inflammatory demyelinating polyradiculoneuropathy associated with T-cell lymphoma. Malignant lymphoma may present prominent peripheral nervous system disorders with variable etiologies. We describe a patient who presented with chronic relapsing polyradiculoneuropathy accompanied by right facial nerve palsy. gadolinium enhancement of the right facial nerve and cervical spinal roots was noted on magnetic resonance imaging (MRI). sural nerve biopsy specimens showed mononuclear cell infiltration around the vessels in the epineurium. Histopathological and immunohistochemical investigations of sural nerve specimens revealed perivascular infiltration of lymphocytes with T-cell dominancy. No apparent direct invasion of lymphoma cells was seen. The results of nerve conduction studies, sural nerve biopsy and cerebrospinal fluid examination were suggestive of immune-mediated inflammatory demyelinating neuropathy. The chronic and relapsing fashion and unique radiological findings in our patient expand on the previously reported features of peripheral neuropathy associated with peripheral T-cell lymphoma. ( info) |