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)

We report a patient with idiopathic hypoparathyroidism associated with unilateral calcification of the basal ganglia and contralateral choreic movements. A 66-year-old woman was admitted to our hospital because of involuntary movements of the left limbs. Two years before the present admission, she had operations for bilateral cataracts. Eight months before admission, she had an onset of severe carpal spasms and pain in both arms and legs. These symptoms improved after one month's rest in a hospital. About a month before admission, she noted progressive difficulty in using her left hand, followed by difficulty in walking due to the abnormal involuntary movement of the left lower limb. Two weeks before admission her carpal spasms appeared again, bilaterally. On admission, she was alert. The general physical examination was normal. Neurological examination revealed choreic movements in the left hand, arm, leg and neck, and grimacing of the left face. She also complained of the spasms and pain in her hands, dysarthria and shortness of the breath. Chvostek and Trousseau signs were positive. Laboratory examination revealed marked decrease in serum calcium level (2.57 mEq/ml) and increase in inorganic phosphorus level (6.40 mEq/ml). Serum level of parathyroid hormone was less than 10 pg/ml. Ellthworth-Howard test was positive, in that the infusion of parathyroid hormone (100 u) elicited a marked increment of the urinary excretions of phosphorus and cAMP. X-ray examination of the spine revealed ossification of the posterior longitudinal ligament in the cervical region. CT-scans of the brain revealed calcification in the region of right globus pallidus and putamen.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Unilateral choreic movements in idiopathic hypoparathyroidism]. 152 May 67

We reported two families each propositus of which exhibited extrapyramidal signs and dementia with bilateral basal ganglia calcifications (BGC), while some of the other non-symptomatic family members showed BCG on brain CT by further examinations. Family 1) A 49-year-old woman was normal until her mid 40s when her memory began to fail. At age 40, dementia, finger-tremor and rigidity were observed and with brain CT and Magnetic Resonance Imaging, BCG and dentate calcifications were found. Her two daughters (20 years old and 26 years old) were free of any neuropsychiatric symptoms, but with CT examinations disclosed BCG. Family 2) A 40-year-old man. His symptoms started at 33 years old. He noticed gradually increasing finger-tremor, rigidity and dysarthria. At 40 years he showed mild dementia and BCG on Brain CT. His mother (64 years old) was non-symptomatic but CT showed that she had BCG. In the two families the calcium, phosphorous and parathyroid hormone levels, and Ellsworth-Howard test were normal. Other specific etiology including infections and somatic abnormalities was not discovered. Familial idiopathic basal ganglia calcification was considered to be rare. But the main purpose of this report is to point out that we must pay attention to the possibility of BCG of non-symptomatic family members if one showed dementia and extrapyramidal signs, and BCG on Brain CT in middle age.
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PMID:[Two cases of familial idiopathic basal ganglia calcifications (BCG) with non-symptomatic family members]. 204 99

We treated a family with idiopathic calcifications of symmetric areas of the brain, including the basal ganglia, dentate, and cerebral white matter. Dementia, progressive dysarthria, incontinence, propulsive-ataxic gait, fixed facies, and cogwheel rigidity without dysmorphic features develop in affected persons. Calcium, phosphorus, and parathyroid hormone levels were normal in the two siblings tested. The literature is reviewed and five other families with a similar syndrome are identified. These six families seem to be clinically distinct from the larger group of idiopathic cerebral calcifications usually referred to as Fahr's disease.
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PMID:Adult onset idiopathic familial brain calcifications. 650 50

Two patients, one with ataxia, internuclear ophthalmoplegia, muscle weakness, atrophy, fasciculations, and bilateral Babinski's signs, the other with dysarthria, dysphagia, muscle weakness, atrophy, fasciculations, and hyperreflexia, had elevated serum calcium and parathyroid hormone levels, establishing the diagnosis of primary hyperparathyroidism (HPT). Removal of a parathyroid adenoma in one patient and three hyperplastic parathyroid glands in the other resulted in remission of the hyperparathyroidism but left both patients with residual neurological damage. Postmortem examination of the second patient showed typical features of amyotrophic lateral sclerosis. The findings in these patients show that hyperparathyroidism may be associated with signs of severe central nervous system disease and that patients with unexplained neurological signs or symptoms should be checked for hyperparathyroidism.
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PMID:Severe neurological disease associated with hyperparathyroidism. 673 92

A 47-year-old man was admitted for evaluation of unsteady gait, postural instability, and dysarthria. On admission, neurological examinations revealed cerebellar ataxia, extrapyramidal signs including parkinsonism and positive Trousseau's sign. Laboratory findings revealed severe hypocalcemia and hyperphosphatemia, and serum intact parathyroid hormone was not detectable. Brain computed tomography revealed severe calcification of basal ganglia and dentate nuclei. He was diagnosed as idiopathic hypoparathyroidism; treatment with 1 alpha (OH) vitamin D3 brought marked improvement of neurological manifestations. We report a rare case of idiopathic hypoparathyroidism presenting with extrapyramidal and cerebellar dysfunction with a review of literature.
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PMID:A rare case of idiopathic hypoparathyroidism with varied neurological manifestations. 868 Jan 1

The case of a 50-year-old woman with cerebral meningioma and concomitant parathyroid adenoma and papillary thyroid carcinoma is presented. She complained of neurological symptoms characterized by right hemiparesis and dysarthria. Cerebral CT and MRI scans revealed a left voluminous frontal parasagittal lesion with the characteristics of a meningioma. Routine laboratory analysis revealed altered values of calcium-phosphorus metabolism. Intravenous infusion of saline solution at 0.9% of NaCl resulted in a reduction of serum ionized calcium. A left craniotomy was performed and a fibroblastic meningioma of 5 cm in diameter was removed. Even though the patient's clinical condition was good, a calcium-phosphorus metabolism test confirmed high plasma levels of ionized calcium and parathyroid hormone. Thyroid and parathyroid ultrasonography revealed multinodular goiter and a parathyroid lesion confirmed by 99mTc-TCO4 / 99mTc-MIBI scintigraphy. A left superior parathyroidectomy and total thyroidectomy were performed. Histological examination revealed a parathyroid adenoma and a small papillary carcinoma of 0.4 cm in the right thyroid lobe. As far as we know, this patient is the third case of meningioma associated with parathyroid adenoma and papillary thyroid carcinoma described in the literature.
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PMID:An unusual association of cerebral meningioma, parathyroid adenoma and thyroid papillary carcinoma. 1672

Fahr's disease occurs in relation with many metabolic disorders especially with hypoparathyroidism. Imbalance of the coordination system and dysarthria were seen at the end of the treatment in a lung cancer patient treated with radiotherapy and chemotherapy. Fahr's disease was diagnosed by diffuse symmetric calcifications at white matter and basal ganglia of cerebrum and cerebellum in cranial computed tomography. Disease was thought to be caused by hypoparathyroidism with lower calcium and parathyroid hormone levels. Possible factor that caused hipoparathyroidism and also of Fahr's disease was radiotherapy performed to a wide area because of lung cancer. This case is the first Fahr's disease that was diagnosed concurrently with lung cancer.
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PMID:[Fahr's disease accompanying to lung cancer]. 2103 45