Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013362 (dysarthria)
3,768 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 59-years-old man with oesophageal cancer (T3NXMo) presented with trismus, dysarthria and diaphoresis. Later, he developed opisthotonus and generalized spasms. Despite negative blood cultures and sufficiently high anti-tetanus-titres, tetanus was suspected, on clinical grounds. He was intubated and treated with tetanus toxoid, human antitetanus immunoglobulin, benzylpenicillin, propofol, benzodiazepines, vecuronium, and sufentanil, and recovered gradually. Tetanus is caused by Clostridium tetani, a Gram-positive rod capable of remaining present latently in the body for years. Absence of a visible external wound suggests that the oesophageal mucosal cancer lesion could have served as portal of entry or that endogenous reactivation of latent tetanus bacteria had taken place.
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PMID:[A patient with tetanus without an obvious point of entry]. 1006 31

Mr. Duffy is 83 years old and is admitted to rehabilitation 4 weeks after a right thalamic cerebral vascular accident (CVA). He has dysphagia, dysarthria, left hemiplegia, and is moderately-severely confused. He pulls out his nasogastric feeding tube and his physician decides not to reinsert it because of significant nasal tissue necrosis. The team recommends a gastrostomy tube for nutrition because of Mr. Duffy's lack of alertness and high risk for aspiration. Mr. Duffy has a Living Will that states he does not wish to have his life sustained with a feeding tube. He does not have a formal Durable Power of Attorney for Health Care. His wife has dementia and their daughters are making decisions for both parents. They are not sure about his wishes in this particular circumstance, but report that he said of a relative who died of cancer, "things went on too long because of that feeding tube." After 3 days, Mr. Duffy is more alert, and during a discussion about tube feedings he says, "I'll go for the works." His fluctuating alertness level prevents him from responding to this question again. His daughters feel he would not want the tube and suggest waiting to see if his swallowing improves in the next week before making a decision.
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PMID:Interpreting a living will after stroke. 1042 Jun 59

Paraneoplastic cerebellar degeneration (PCD) is the most frequently seen paraneoplastic syndrome affecting the brain. PCD is most commonly associated with cancers of the ovary, breast, and lung. The anti-Purkinje cell antibodies (anti-Yo) that specifically damage the Purkinje cells of the cerebellum are found in the patient's serum and cerebrospinal fluid. The typical presentation of PCD includes limb and truncal ataxia, often along with dysarthria. This report describes the case of a 47-year-old woman without significant medical history who developed new onset of unsteady gait, headache, and vertigo. The imaging studies suggested rhombencephalitis. The patient initially responded to corticosteroid treatment. Unfortunately, her gait ataxia worsened and she developed dysarthria, neither of which responded to increasing dosages of corticosteroids. Extensive imaging studies showed no evidence of tumor, but the patient was found to have positive anti-Yo antibodies and elevated cancer antigen 125 (CA-125). Pathology results from exploratory laparotomy revealed stage III C adenocarcinoma of the ovary. This case demonstrates that PCD may be the presenting symptom of an occult malignancy. The pathogenesis, diagnosis, and treatment of PCD, and its rehabilitation implications, are reviewed.
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PMID:Paraneoplastic cerebellar degeneration as the first evidence of cancer: a case report. 1085 34

Laparoscopic adrenalectomy has become increasingly popular because of its minimally invasive nature, but guidelines for selection of cases suitable for this surgical procedure have not been established. We report a 52-year-old woman with adrenocortical carcinoma, manifesting as Cushing's syndrome, treated with laparoscopic adrenalectomy. The tumour was removed in toto and had been histologically diagnosed as adrenocortical adenoma. However, the patient developed intra-abdominal peritoneal dissemination of carcinoma 15 months after surgery. Review of the histopathological findings of the resected adrenocortical tumour revealed that the neoplasm met five out of nine histological criteria for adrenocortical malignancy, and was diagnosed as adrenocortical carcinoma. Histopathological examination of the tumour was also consistent with adrenocortical carcinoma. The patient responded extremely well to chemotherapy, including carboplatin, etoposide and o,p'-DDD (1,1-dichlorodiphenyldichloroethane), and a subsequent CT (computed tomography) scan 12 months after the start of chemotherapy demonstrated no evidence of disease. However, the patient developed neurological impairment, including dysarthria, as a side-effect of o, p'-DDD. The patient died of aspiration pneumonia due to a decreased pharyngeal reflex. Postmortem examination revealed no foci of residual carcinoma. This case report emphasizes the importance of excluing possible adrenocortical malignancy in patients considered for laparoscopic adrenalectomy, histopathological diagnosis of adrenocortical malignancy and careful monitoring for neurotoxicity during o,p'-DDD treatment.
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PMID:A case of adrenocortical carcinoma associated with recurrence after laparoscopic surgery. 1093 Nov 7

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.
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PMID:Successful immunosuppressant therapy of severe progressive cerebellar degeneration and sensory neuropathy: a case report. 1101 51

Paraneoplastic cerebellar degeneration (PCD) is a type of paraneoplastic syndrome that primarily affects women with gynecological cancers. Patients typically experience pancerebellar symptoms, including gait ataxia, dysarthria, nystagmus, and truncal and appendicular ataxia. We present the case of a 50-year-old woman with PCD and presumed ovarian cancer who initially complained of ataxia and dysarthria. PCD was diagnosed on the basis of her symptoms, diagnostic imaging, and laboratory work. PCD symptoms may precede the diagnosis of malignancy by months or years. Early diagnosis and treatment of these syndromes, including rehabilitation, may result in improvements in quality of life for this population of patients.
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PMID:Paraneoplastic cerebellar degeneration as the first manifestation of cancer. 1144 49

Friedreich's ataxia (FRDA) is an autosomal recessive neurodegenerative disorder causing progressive ataxia and dysarthria. We report two sisters who had breast cancer aged 39 years and 42 years and who both developed a late onset form of FRDA with onset of neurological symptoms in their thirties. We discuss whether there may be an association between the late onset form of FRDA and malignancy.
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PMID:Breast cancer in two sisters with Friedreich's ataxia. 1150 27

Primary malignant melanoma of the leptomeninges is a rare and aggressive tumor in children and accounts for less than 1% of all pediatric malignancies. Usually its symptoms include raised intracraneal pressure resulting from hydrocephalus secondary to tumoral obliteration of basal cisterns, but the passage of time from the initial symptomatology to diagnosis is frequently delayed. A 7-year-old male with primary leptomeningeal melanoma is reported. At the beginning, he presented ataxia and dysarthria followed by symptoms of raised intracranial pressure, complex partial seizures, progressive loss of consciousness, and coma. Cerebrospinal fluid analysis demonstrated raised opening pressure, normal glucose, and increased protein concentration, but malignant melanoma cells were not found. Magnetic resonance imaging scans depicted bright signals in the subarachnoid spaces on T(1) images and gadolinium-enhanced focal lesions. Cerebral biopsy was proposed, but it was not authorized. Definitive diagnosis was thus made by pathologic postmortem examination.
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PMID:Primary leptomeningeal melanoma in a child. 1151 18

A 54-year-old female with small volume residual stage III ovarian cancer had received two courses of carboplatin chemotherapy with obvious response, when she developed rapidly progressive neurological symptoms. Over a period of 48 h, an incapacitating syndrome of ataxia, nystagmus and dysarthria evolved. Central nervous system metastases were excluded by computed tomography scanning and cerebrospinal fluid cytology. Anti-Purkinje cell antibodies ('anti-Yo') detected in the serum confirmed the diagnosis of paraneoplastic cerebellar degeneration. Isolated reports have suggested that the clinical course of this condition can be ameliorated with high dose steroids and plasmapheresis. However, in this case the very early introduction of both these did not bring about any improvement in the patient's symptoms. She remained severely incapacitated and unable to care for herself for the remaining 15 months of her life. The patient died of progressive ovarian cancer that had become clinically evident 10 months after the onset of neurological symptoms. This case illustrates many of the classical features of this rare condition, and the world literature is reviewed.
Int J Gynecol Cancer 1995 Sep
PMID:Paraneoplastic cerebellar degeneration in a patient with chemotherapy-responsive ovarian cancer. 1157 11

Paraneoplastic cerebellar degeneration (PCD) is a heterogeneous group of disorders characterized by subacute cerebellar ataxia, specific tumour types and (often) associated antineuronal antibodies. Nine specific antineuronal antibodies are associated with PCD. We examined the relative frequency of the antineuronal antibodies associated with PCD and compared the neurological symptoms and signs, associated tumours, disability and survival between groups of PCD with different antibodies. Also, we attempted to identify patient-, tumour- and treatment-related characteristics associated with functional outcome and survival. In a 12-year period, we examined >5000 samples for the presence of antineuronal antibodies. A total of 137 patients were identified with a paraneoplastic neurological syndrome and high titre (> or =400) antineuronal antibodies. Fifty (36%) of these patients had antibody-associated PCD, including 19 anti-Yo, 16 anti-Hu, seven anti-Tr, six anti-Ri and two anti-mGluR1. Because of the low number, the anti-mGluR1 patients were excluded from the statistical analysis. While 100% of patients with anti-Yo, anti-Tr and anti-mGluR1 antibodies suffered PCD, 86% of anti-Ri and only 18% of anti-Hu patients had PCD. All patients presented with subacute cerebellar ataxia progressive over weeks to months and stabilized within 6 months. The majority of patients in all antibody groups had both truncal and appendicular ataxia. The frequency of nystagmus and dysarthria was lower in anti-Ri patients (33 and 0%). Later in the course of the disease, involvement of non-cerebellar structures occurred most frequently in anti-Hu patients (94%). In 42 patients (84%), a tumour was detected. The most commonly associated tumours were gynaecological and breast cancer (anti-Yo and anti-Ri), lung cancer (anti-Hu) and Hodgkin's lymphoma (anti-Tr and anti-mGluR1). In one anti-Hu patient, a suspect lung lesion on CT scan disappeared while the PCD evolved. Seven patients improved by at least 1 point on the Rankin scale, while 16 remained stable and 27 deteriorated. All seven patients that improved received antitumour treatment for their underlying cancer, resulting in complete remission. The functional outcome was best in the anti-Ri patients, with three out of six improving neurologically and five were able to walk at the time of last follow-up or death. Only four out of 19 anti-Yo and four out of 16 anti-Hu patients remained ambulatory. Also, survival from time of diagnosis was significantly worse in the anti-Yo (median 13 months) and anti-Hu (median 7 months) patients compared with anti-Tr (median >113 months) and anti-Ri (median >69 months). Patients receiving antitumour treatment (with or without immunosuppressive therapy) lived significantly longer [hazard ratio (HR) 0.3; 95% confidence interval (CI) 0.1-0.6; P = 0.004]. Patients > or =60 years old lived somewhat shorter from time of diagnosis, although statistically not significant (HR 2.9; CI 1.0-8.5; P = 0.06).
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PMID:Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patients. 1276 61


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