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 58-year-old female was admitted to our hospital because of orthostatic syncope, and difficulty in urination and walking for 2 years. At age 35, she suffered from cerebral hemorrhage and was diagnosed as Moyamoya disease (MD). Neurological examination on admission revealed severe orthostatic syncope so that she could not stand. At that time, her blood pressure and heart rate were 104/74 mmHg and 78/min in supine position and 52/48 mmHg and 52/min in 60 degrees head-up position. She also had severe pollakisuria, cerebellar ataxia and dysarthria. Cranial nerves, motor strength and deep reflexes were normal. ECG, chest X-ray, and EEG were normal. Aschner, Czermak, and cold pressor tests revealed no response. Urodynamic study revealed autonomic bladder. MRI showed only enlarged fourth ventricle because of atrophy of the pons. Bilateral CAG revealed "Moyamoya" vessels in the cerebral basal regions. Cerebral blood flow (CBF) was measured by 133Xe inhalation method. Mean arterial blood pressure changed from 134 mmHg to 126 mmHg in 45 degrees head-up tilting and CBF decreased from 47.5 mg/100 g/min to 37 ml/100 g/min position. Though there was no relationship between SDS and MD, each one shows dysautoregulation of CBF, it is supposed that a severe orthostatic syncope attack was resulted from synergism of both effects.
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PMID:[An association of Shy-Drager syndrome with moyamoya disease--a case report]. 240 Nov 16

We report a 54-year-old man with progressive proximal muscle atrophy and gynecomastia. The patient had an insidious onset of weakness in his lower extremities at age 14, in that he noted a difficulty in standing up from a chair. Soon after he noted some difficulty in climbing up stairs. At age 35, he noted weakness in his arms; his weakness slowly progressed in that he became unable to walk or stand alone before 40 years of age. He also noted gynecomastia at that age. He was admitted to our hospital for the work up on September 16, 1993, when he was 54-year-old. On admission, he was alert and oriented; his BP was 150/70 mmHg; he had bilateral gynecomastia, however, no other skeletal deformities were found. On neurologic examination, he was mentally sound without dementia, and his higher cerebral functions were normal. Cranial nerves also appeared intact without facial atrophy, dysarthria, or dysphagia; no atrophy was noted in the tongue. He had marked muscle atrophy in both upper and lower extremities more marked in the proximal portions; muscle strength was approximately in the range of 2/5 to 3/5 in the proximal parts, and 4/5 in the distal parts in both upper and lower extremities. No fasciculation was noted; muscle tone was flaccid; no ataxia was present. Deep reflexes were either lost or markedly diminished. No Babinski sign was noted. Sensation was intact. Laboratory examination revealed normal blood counts; serum CK was slightly increased to 131 IU/l; ECG showed complete right bundle branch block; EMG revealed no active units in the right biceps brachii, deltoid, quadriceps femoris, and triceps surae muscles; in other muscles tested, motor unit potentials of low amplitude and short duration were seen; in the right tibialis anterior muscle, however, motor unit potentials with an amplitude up to 6 m V were also seen. Nerve conduction velocities were normal. A diagnostic procedure was performed. He was discussed in the neurological CPC, and the chief discussant arrived at the conclusion that this patient had Becker type of progressive muscular dystrophy. In her differential diagnosis, the possibility of Kennedy-Alter-Sung syndrome was discussed because this patient had gynecomastia. However, the discussant excluded that possibility because of absence of both bulbar symptoms and typical neurogenic changes in his EMG. The diagnostic procedure was a muscle biopsy on the left tibialis anterior muscle. Histologic observation on HE stained specimens revealed marked inequality in the muscle fiber diameters, increase in endomysial nuclei, proliferation of connective tissue, and fiber splitting.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[A 54-year-old man with progressive proximal muscle atrophy and gynecomastia]. 766 8

A 65-year-old hypertensive man was admitted because of abnormal involuntary movements which had an onset one month prior to the admission. Neurological examination revealed an alert and intelligent male in no acute distress. Cranial nerves appeared intact except for dysarthria. His gait was wide-based. He had hemiballism-hemichorea in his left upper and lower extremities. Cranial CT scans revealed a high density area confined to the right putamen. After admission, his involuntary movements slowly improved without medication. After near complete disappearance of his abnormal movements two weeks after admission, he noted that smoking induced choreic movement in his left side transiently. The involuntary movement ceased soon after upon stopping smoking. This was repeatedly observed not only by him but also by us. Our observation may indicate that the inhibition of the subthalamic nucleus as the result of the decrease of the putaminal output to the external segment of the globus pallidus will induce hemiballism. The effect of smoking on his involuntary movements can be understood as a result of increase in the dopamine release from the nigrostriatal terminals induced by nicotine.
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PMID:[Hemiballism-hemichorea caused by a putaminal hemorrhage with worsening after smoking--a case report]. 836 67

We report a 63-year-old man with progressive gait disturbance and dysarthria. The patient was apparently well until the age of 62 (February, 1990) when he noted unsteadiness of gait. Two months later, dysarthria appeared. He was admitted to Juntendo Izunagaoka Hospital on April 23, 1990. Neurologic examination revealed a mentally sound man with normal higher cerebral functions. Cranial nerves were unremarkable except for scanning speech. His gait was ataxic with positive Romberg sign. No motor weakness was noted, however, he had hypotonia and cerebellar ataxia. Deep tendon reflexes were retained and the plantar response was flexor. Pain, touch and vibration senses were diminished in the distal parts of the lower extremities. Laboratory examination revealed a 2.5 cm mass in the left lung field. Cranial MRI revealed a small T1-low and T2-high signal intensity lesion in the left temporal lobe. Abdominal CT scan revealed multiple low density lesions in the liver. His subsequent course was complicated by progressive deterioration in his gait and loss of deep tendon reflexes. He expired on November 24, 1990. The patient was discussed in the neurological CPC and the chief discussant arrived at the conclusion that the patient had anti-Hu associated paraneoplastic encephalomyelitis and sensory neuropathy. Some other participants thought that the patient had carcinomatous cerebellar degeneration. Postmortem examination revealed a 4x4 cm mass lesion involving the left S4-S5 segments. Histologic examination of the tumor was small cell carcinoma. Many metastatic foci were found in the liver. The cerebral hemispheres were unremarkable except for a small wedge-shaped tissue defect in the left temporal lobe which appeared to have been caused by old head trauma which the patient had received. The cerebellar vermis showed slight enlargement of cortical sulci, however, the cerebellar hemispheres appeared unremarkable. Upon histologic examination, marked loss of Purkinje cells was noted, particularly in the cerebellar anterior lobe. The dentate nucleus showed slight cell loss with increase in fat granule cells. The inferior olive was normal. The histologic characteristics were consistent with the pathologic diagnosis of carcinomatous cerebellar degeneration. No evidence of limbic encephalitis was seen. The peripheral nerve was not examined.
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PMID:[A 63 year-old man with progressive gait disturbance and dysarthria]. 888 38

Gliomas are rare entities in the cerebellopontine angle (CPA) in adults. The authors present clinical, neuroradiological, serological, and neuropathological findings in a 60-year-old man with an extraaxial CPA glioblastoma arising from the proximal portion of cranial nerve VIII. The patient presented with progressive left-sided deafness and left-sided facial palsy lasting less than 2 months and progressive dysarthria and dysphagia lasting 2 weeks. Preoperative neuroimaging suggested the diagnosis of CPA meningioma with "dural-tail" sign and involvement of the internal auditory canal. Serological examination showed an increase in the malignant markers of ferritin and neuron-specific enolase, which suggested underlying malignancy. The tumor was subtotally removed, and it was confirmed to be completely separated from the brainstem and cerebellum. Cranial nerves VII and VIII were destroyed and sacrificed. Transient severe bradycardia occurred during surgery due to entrapment of the caudal cranial nerve complex by the tumor in such an infiltrative way. The neuropathological examination revealed a glioblastoma. The patient underwent no further treatment and died of cachexia 2 months postoperatively. To the authors' knowledge, this represents the first case of a primary glioblastoma in the CPA in an adult. A high index of suspicion along with reliance on clinical assessment, radiological findings, and serum detection of specific malignant markers is essential to diagnose such uncommon CPA lesions.
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PMID:Primary glioblastoma of the cerebellopontine angle in adults. 2178 Aug 57