1/11. 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.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/11. paraneoplastic polyneuropathy preceding the diagnosis of Hodgkin's disease and non-small cell lung cancer in a patient with concomitant borrelia burgdorferi infection.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.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
3/11. 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.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/11. 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.- - - - - - - - - - ranking = 2.25keywords = nerve (Clic here for more details about this article) |
5/11. 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.- - - - - - - - - - ranking = 1.5keywords = nerve (Clic here for more details about this article) |
6/11. Small cell lung cancer associated with anti-Hu paraneoplastic sensory neuropathy and peripheral nerve microvasculitis: case report and literature review.A 53-year-old man presented with an 8-week history of upper and lower limb paraesthesia. Neurological examination revealed a glove and stocking distribution of sensory loss. sural nerve biopsy showed severe axonal neuropathy associated with microvasculitis. positron-emission tomography and thoracic computed tomography helped in localising the underlying malignancy. A transbronchial biopsy confirmed the diagnosis of small cell lung carcinoma (SCLC). Neuroimmunological studies identified anti-Hu antibodies and confirmed a paraneoplastic aetiology for his neuropathy. Treatment of small cell lung cancer with carboplatin and etoposide resulted in significant improvement of neurological symptoms. We report a case of a patient with SCLC and anti-Hu paraneoplastic sensory neuropathy with microvasculitis, and discuss the literature on prognosis of patients with SCLC with paraneoplastic neurological syndromes compared with patients with SCLC only.- - - - - - - - - - ranking = 1.25keywords = nerve (Clic here for more details about this article) |
7/11. Anti-hu paraneoplastic syndrome presenting as bilateral sixth cranial nerve palsies.A 62-year-old woman presented with diplopia caused by bilateral sixth cranial nerve palsies. Two weeks later, she had bulbar weakness and ataxia. Brain magnetic resonance imaging showed non-specific abnormalities and spinal fluid was acellular but contained an elevated protein and oligoclonal bands. A paraneoplastic screen showed anti-Hu antibodies. Her clinical condition improved with immunoglobulin and systemic corticosteroid treatment. breast cancer was diagnosed 21 months later by mammography but there were no metastases detected. Four and half years after the onset of her diplopia, she died of diffuse metastatic breast cancer. This is the first reported case of anti-Hu paraneoplastic brain stem encephalitis presenting with sixth cranial nerve palsies.- - - - - - - - - - ranking = 1.5keywords = nerve (Clic here for more details about this article) |
8/11. Neuropathology of paraneoplastic neuropathy with anti-disialosyl antibody.We report a paraneoplastic neuropathy with severe motor involvement following sensory-ataxic disturbance. Anti-disialosyl immunoglobulin m (IgM) antibody was detected in the course of malignant lymphoma of diffuse large B-cell type, which usually spares the motor system. Onset was subacute, with relapsing and remitting sensory ataxia, muscle weakness, bulbar palsy, respiratory paralysis, and ophthalmoplegia; only neck rotation was retained in the terminal stage. autopsy showed no lymphoma cells infiltrating the nervous system. motor neurons survived in the spinal cord, but mean diameter of the ventral spinal nerve roots was reduced considerably. The gracile fasciculus and the sural nerve were more markedly degenerated than proximal portions. Morphometric study showed that most of the proximal motor and sensory axons did not extend distally. This autopsy report provides further definition of a neuropathy associated with malignant lymphoma and IgM antibodies against disialosyl residues.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
9/11. Impairment of Trk-neurotrophin receptor by the serum of a patient with subacute sensory neuropathy.BACKGROUND: Paraneoplastic peripheral neuropathy is sometimes associated with unidentified neuronal autoantibodies. OBJECTIVE: To examine the effects of serum from a patient with subacute sensory axonopathy on the function of the Trk high-affinity nerve growth factor receptor. PATIENT: An 86-year-old man with sensory neuropathy exhibiting an autoantibody to Trk. methods: Immunoblot analyses of the brain homogenates and immunoprecipitation were performed with human sera. We further examined the effect of sera on nerve growth factor-induced neurite outgrowth and Trk autophosphorylation. RESULTS: The patient showed sensory nerve axonopathy without well-known paraneoplastic autoantibodies. His serum inhibited nerve growth factor-induced neurite outgrowth and Trk autophosphorylation in PCtrk cells. Moreover, the patient's serum, but not control serum, immunoprecipitated Trk and recognized Trk in brain homogenates as well as in Trk immunoprecipitates. CONCLUSION: These data strongly suggest that an anti-Trk autoantibody might cause subacute sensory neuropathy.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
10/11. Paraneoplastic mononeuritis multiplex in non-small-cell lung carcinoma.A 60-year-old man developed two selective peripheral mononeuropathies of the peroneal and later the radial nerve, shortly after a diagnosis of large-cell lung carcinoma. Nerve conduction studies and electromyography confirmed isolated lesions in both nerves, and in the case of the peroneal nerve lesion, focal conduction block was localised to the level of the fibula neck. Subsequent magnetic resonance imaging of the lower limb excluded focal compression or malignant infiltration along the course of the peroneal nerve, and there was no signal change within the nerve, prompting a diagnosis of paraneoplastic mononeuritis multiplex. Anti-neuronal antibodies and serological markers of systemic vasculitis were negative. Neither the patient's large-cell lung carcinoma nor mononeuritis multiplex responded to chemotherapy, and he died within 6 months of the initial diagnosis.- - - - - - - - - - ranking = 1.25keywords = nerve (Clic here for more details about this article) |
| | Next -> |