1/100. Antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors.BACKGROUND: Antiamphiphysin antibodies react with a 128-kd protein found in synaptic vesicles.They were first described in patients with paraneoplastic stiff-man syndrome and breast cancer, but studies suggest that they can also occur in patients with other tumors and neurological disorders. OBJECTIVE: To determine if antiamphiphysin antibodies are associated with various paraneoplastic neurological syndromes and tumors. patients AND methods: Of 2800 serum samples tested by routine immunohistochemical procedures on sections of paraformaldehyde-fixed rat brain for the detection of autoantibodies associated with paraneoplastic neurological syndromes, 5 were selected because of labeling suggestive of antiamphiphysin antibodies and subsequently confirmed by the results of Western blot analysis using recombinant amphiphysin protein. Controls consisted of 40 patients with various nonparaneoplastic neurological diseases; 101 patients with cancer but without paraneoplastic neurological syndrome; 9 patients with small cell lung cancer, anti-Hu antibodies, and paraneoplastic neurological syndrome; 3 patients with M2-type antimitochondrial antibodies but no neurological disorder; and 30 normal subjects. RESULTS: Of the 5 patients with antiamphiphysin antibodies, patient 1 had sensory neuronopathy, encephalomyelitis, and breast cancer; patient 2 had limbic encephalitis, and small cell lung cancer was detected in the mediastinum after 24 months of follow-up; patient 3 had encephalomyelitis and ovarian carcinoma; and patients 4 and 5 had lambert-eaton myasthenic syndrome and small cell lung cancer (patient 4 subsequently developed cerebellar degeneration). None of the 5 had stiffness. Two patients (Nos. 2 and 4) had antimitochondrial antibodies. The two patients (Nos. 4 and 5) with lambert-eaton myasthenic syndrome had antibodies directed against the voltage-gated calcium channel, and patient 2 subsequently developed anti-Hu antibodies. In the controls, antiamphiphysin antibodies were detected by Western blot analysis in 3 of 8 patients with anti-Hu antibodies, but in none of the other groups. CONCLUSIONS: These data indicate that antiamphiphysin antibodies are not specific for one type of tumor or one neurological syndrome and can be associated with other neural and nonneural antibodies. The simultaneous association of several antibodies in some patients suggests multimodal autoantibody production.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/100. Neuro-ophthalmologic manifestations of a paraneoplastic syndrome and testicular carcinoma.The authors report two patients with testicular cancer who exhibited supranuclear gaze disorders as a manifestation of a paraneoplastic brainstem encephalomyelitis. In the first patient, neuro-ophthalmic dysfunction was accompanied by a prominent limbic encephalitis whereas in the second patient, an unusual, mixed pendular and jerk nystagmus was manifested. neuroimaging revealed an enhancing hypothalamic mass in the first patient and was negative in the second. blood from both patients contained an antibody previously reported in a patient with limbic encephalitis and testicular cancer.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
3/100. A case of the subacute brainstem encephalitis.A case of brainstem encephalitis of undetermined etiology is reported in 66-year-old woman who had a sudden onset of illness with left abducens palsy, nystagmus and ataxia. The symptoms progressed to complete paralysis of eye movements, dysphagia and left hemiparesis with generalized hyperreflexia. Examination of CSF, CT scan and MRI of the brain were normal. The patient died 4 months after onset of disease. Neuropathologic study disclosed in the brainstem numerous perivascular and nodular inflammatory cell infiltrations composed predominantly of lymphocytes T and B. Most intensive inflammation concerned midbrain and pontine tegmentum and to a lesser degree medulla oblongata, pontine nuclei and cerebellar nuclei. basal ganglia, cerebral and cerebellar cortex were unaffected. Neuropathological finding was reminiscent of brainstem encephalitides related to viral infection or to paraneoplastic syndrome. However, HSV-1, EBV, and CMV antigens were not detected by immunohistochemistry, as well as evidences of malignancy were not present in this case.- - - - - - - - - - ranking = 9keywords = brain (Clic here for more details about this article) |
4/100. Paraneoplastic limbic encephalitis associated with small cell carcinoma of the prostate.A 76-year-old man with primary small cell carcinoma of the prostate died after a subacute illness marked by memory loss and truncal ataxia Post-mortem examination of the central nervous system was consistent with limbic encephalitis and cerebellar degeneration. Although limbic encephalitis is a known complication of small cell carcinoma of the lung, this seems to be the first reported case of limbic encephalitis associated with small cell carcinoma of the prostate. Implications with respect to diagnosis and therapy are discussed.- - - - - - - - - - ranking = 8.3328388872009keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/100. Reversible paraneoplastic encephalomyelitis associated with a benign ovarian teratoma.BACKGROUND: Paraneoplastic encephalomyelitis (PEM) is a well characterized, and typically irreversible, paraneoplastic syndrome, usually associated with small cell lung cancer or other malignancy. We describe a case of a young woman with a benign ovarian teratoma who presented with a reversible PEM. CASE REPORT: A 24-year-old woman presented with a three week history of memory impairment, unusual behavior, personality changes, auditory hallucinations, hypersomnolence and binocular biplopia. On admission she was disoriented and inattentive with impaired short term memory. Small doses of lorazepam (1 mg), given for episodic agitation, repeatedly induced multidirectional bilateral nystagmus and a skew deviation, but her neurological examination was otherwise normal. A left-sided pelvic mass was palpable. brain MRI pre- and post-gadolinium was normal. There was a mild CSF pleocytosis and an EEG showed minimal bilateral background activity irregularities. There were no other laboratory abnormalities. Two weeks after admission, she clinically deteriorated developing central respiratory failure and a flaccid paraplegia. Repeat MRI showed an area of increased T2 weighted signal in the medulla and three similar areas in the spinal cord. Following removal of her tumor, treatment with high dose corticosteroids and intravenous immunoglobulin, she ultimately made a full recovery. pathology revealed the tumor to be a benign ovarian cystic teratoma. CONCLUSIONS: This is the first report of a reversible PEM seen in association with a benign tumor, in this case a mature ovarian teratoma. Presumably, an immune response directed against neural clements of the teratoma cross-reacted with normal brain, brainstem and spinal cord antigens to cause neurologic symptoms. Tumor removal was followed by neurologic recovery.- - - - - - - - - - ranking = 2keywords = brain (Clic here for more details about this article) |
6/100. Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature.OBJECTIVE AND IMPORTANCE: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis. CLINICAL PRESENTATION: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe. INTERVENTION: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission. CONCLUSION: Previous reports of central nervous system angiitis have shown an association with sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.- - - - - - - - - - ranking = 66.662711097607keywords = central nervous system, nervous system (Clic here for more details about this article) |
7/100. Craniofacial hemangiopericytoma associated with oncogenic osteomalacia: case report.A craniofacial hemangiopericytoma associated with oncogenic osteomalacia is described and the literature is reviewed. A 46 year-old male with multiple fractures and hypophosphatemia was found to have a craniofacial mass extending from the right ethmoid sinus into the right frontal lobe. Initial detection of the tumor was made with an 111Indium-pentreotide scan (Octreoscan). Gross total resection of the tumor was achieved and the patient received postoperative radiation therapy. One year after surgery, the patient remains free of tumor with significant increase in bone density and normal phosphate levels. This is the first report of a hemangiopericytoma invading the brain that was associated with paraneoplastic hypophosphatemia and osteomalacia. Also, this is the first reported detection of a hemangiopericytoma by an Octreoscan. Primary detection and secondary surveillance of hemangiopericytomas may be possible with serial Octreoscans.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
8/100. Chronic multiple paraneoplastic syndromes.A patient presented with symptoms of limbic and brainstem encephalitis, motor and sensory neuronopathy, cerebellar dysfunction, and highly positive anti-Hu antibodies. He also harbored P/Q-type calcium channel antibodies and manifested the lambert-eaton myasthenic syndrome (LEMS). Small-cell lung cancer was found, and he received both antineoplastic therapy and intravenous immunoglobulin (IVIg). Remission of the malignancy was achieved. Although the anti-Hu-related manifestations improved after therapy, LEMS has persisted, leading to IVIg dependency.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
9/100. Paraneoplastic and metastatic neurologic complications of Merkel cell carcinoma.Merkel cell carcinoma is a rare primary cutaneous neuroendocrine tumor that is locally aggressive and frequently accompanied by distant metastases. Neurologic complications of Merkel cell carcinoma are rare. We describe a 69-year-old man who presented with lambert-eaton myasthenic syndrome and was found to have Merkel cell carcinoma. The paraneoplastic syndrome improved with initial treatment of the malignancy. He subsequently developed a solitary brain metastasis and died of leptomeningeal carcinomatosis.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
10/100. Ballistic-choreic movements as the presenting feature of renal cancer.BACKGROUND: The paraneoplastic syndromes can involve multiple areas of the central nervous system and result in a variety of neurological symptoms. To our knowledge, severe, rapidly progressive, and drug-resistant ballistic-choreic movements have not been previously described as the presenting feature of renal cell carcinoma. PATIENT AND methods: A previously healthy 55-year-old man developed limb ballismus and involuntary choreic movements of his face over several weeks. Extensive laboratory, diagnostic, and radiographic studies failed to reveal a cause, until an abnormality on a chest x-ray film prompted a search for a primary neoplasm and a final diagnosis of renal cell carcinoma. High doses of medications traditionally used to treat choreic disorders had no effect on the abnormal movements. A biopsy specimen of the basal ganglia showed focal encephalitic changes but no malignant neoplasm. CONCLUSIONS: Whereas prior cases of paraneoplastic syndromes with chorea have been reported in other forms of cancer, our case was significant because, to our knowledge, renal cell carcinoma has not been previously reported in association with this syndrome. Furthermore, the chorea was categorically resistant to pharmacological treatment, and the movement disorder was the initial and only focal neurological feature of the primary illness.- - - - - - - - - - ranking = 8.3328388872009keywords = central nervous system, nervous system (Clic here for more details about this article) |
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