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 32 year old male is described with an onset of upper limb postural tremor in adolescence followed by muscle cramps. Progressive proximal amyotrophy and weakness in the limbs developed late in the third decade. Examination disclosed, in addition, bilateral facial weakness and mild dysarthria. Enzyme studies revealed hexosaminidase A and B deficiency, indicating a diagnosis of Sandhoff disease. Intra-axonal membranocytoplasmic bodies were present in a rectal biopsy. The presentation, which resembled that of X-linked bulbospinal neuronopathy, widens the clinical spectrum for disorders related to G(M2) gangliosidosis.
J Neurol Neurosurg Psychiatry 1989 Sep
PMID:Sandhoff disease mimicking adult-onset bulbospinal neuronopathy. 279 83

The case of a female patient with infantile onset of progressive dystonia, disturbance of gait and dysarthria is presented. At age 7, the diagnosis of Hallervorden-Spatz disease was established by clinical findings including retinal pigment degeneration, basal ganglia hyperdensity on CT, and the rare association of acanthocytosis. The clinical course was followed over 15 years until the patient's death.
Monatsschr Kinderheilkd 1989 Sep
PMID:[Hallervorden-Spatz syndrome with acanthocytosis]. 281 85

A 42-year-old man presented with right temporal headache, dysarthria, and dysphagia. On examination, he had a right hypoglossal nerve palsy. The diagnosis of right internal carotid artery dissection was suggested by magnetic resonance imaging and confirmed by carotid angiography. A dynamic computed tomogram demonstrated enlargement of the carotid artery. In carotid dissection, the hypoglossal nerve may be compromised by local factors as it passes close to the carotid artery in the neck.
Stroke 1988 Sep
PMID:Spontaneous internal carotid artery dissection presenting as hypoglossal nerve palsy. 304 72

Iterative dysarthria is a speech disorder which bears some resemblance to stuttering. It is commonly found in Parkinson's disease but has so far received little attention to. We report the case of a 67 year old female parkinsonian patient with a severe dysarthria of the iterative type. The characteristic features of iterative dysarthria are described and the relevant literature is reviewed.
Nervenarzt 1988 Sep
PMID:[Iterative dysarthria in Parkinson disease]. 305 96

Twin males aged 24 years showed dementia, dysarthria, gait disturbances and involuntary movements, with slightly low levels of serum copper and ceruloplasmin, and markedly low excretion of urinary copper. We propose that the unique combination of dementia, dysarthria, gait disturbances, involuntary movements and abnormalities of copper metabolism does not fit any known nosological entity and constitutes a "new" syndrome different from Wilson's and Menkes' diseases.
J Neurol 1988 Sep
PMID:An unusual neurological disorder with abnormal copper metabolism. 306 63

The authors presented a surgically treated case of cavernous angioma extending from the ventral part of the pons to the midbrain. A 20-year old man was admitted to our service with left motor weakness, dysarthria and consciousness disturbance. CT scan revealed a round well-circumscribed high density lesion in the pons and midbrain. This was diagnosed preoperatively as a cryptic vascular malformation with hemorrhage. The right temporal craniotomy with keel form skin incision and subtemporal & trans-tentorial approach was performed in an attempt to remove the hematoma and the angioma. Histological examination of the surgical specimen revealed abnormal vessels as a cavernous angioma. The postoperative course was uneventful.
No Shinkei Geka 1988 Sep
PMID:[A case of cavernous angioma extended from the ventral part of the pons to the midbrain: subtemporal and trans-tentorial approach]. 320 61

The motor score with and without levodopa was estimated in 193 parkinsonian patients with variable length of evolution. The effect of levodopa on akinesia, rigidity, and tremor remained quite stable during the course of the disease. In contrast, the aggravation of gait disorder, postural instability, and dysarthria was more severe, with decreased percentage of improvement on levodopa in patients with longer evolution. It is suggested that aggravation of Parkinson's disease mainly results from increasing severity of cerebral nondopaminergic lesions.
Neurology 1987 Sep
PMID:Does long-term aggravation of Parkinson's disease result from nondopaminergic lesions? 362 54

Communicating syringes confined to the brain stem are extraordinarily rare. Two patients, presenting with signs and symptoms of cerebellar dysfunction, later developed evidence of brain-stem disease with dysarthria, nystagmus, deafness, and internuclear ophthalmoplegia. The condition of both patients had been diagnosed clinically as multiple sclerosis, but at autopsy they had a striking keyhole-shaped syrinx in the midbrain and upper pons, which communicated with the aqueduct and fourth ventricle without associated syringomyelia. In addition, both patients had marked atrophy and gliosis of the cerebellum, one with extension of the syrinx into cerebellar folia. The unique character of these lesions coupled with the similarity of the clinical features of the cases prompted us to name this disorder--"keyhole aqueduct syndrome."
Arch Neurol 1986 Sep
PMID:Keyhole aqueduct syndrome. 374 Dec 9

A case of cardiac myxoma presenting as metastatic brain tumor are reported. The patient was a 44-year-old man. One year prior to this admission, he had suffered stroke, which was characterized by right hemiparesis and dysarthria. The computed tomographic (= CT) scan of the head at that time showed a low density on the left basal ganglia and the echocardiogram suggested a left atrial myxoma. At surgery, a polypoid myxoma attached to the atrial septum was totally removed. Right hemiparesis was improved and the patient was discharged. A few months later, the patient was evaluated for multiple cutaneous masses and diagnosed by biopsy as metastatic myxoma. The patient's condition remained unchanged until this admission. In March 1985, the patient had a tonic-clonic convulsion marching from right hand and developed right hemiplegia with drowsy. An echocardiogram failed to reveal recurrence of the cardiac myxoma. A CT scan revealed a 5-cm, relatively circumscribed, low density mass in the left fronto-parietal lobe, ring mottled enhancement after contrast administration and more enhancement in the delayed scanning of 45 min. Craniotomy showed a tender, friable tumor with a yellowish cyst fluid, but apparently not invading the brain parenchyma. After complete excision of the mass, there was rapid lessing in the hemiplegia and improvement in the level of consciousness. A contrast-enhancement CT scan performed 2 weeks after craniotomy revealed no evidence of residual tumor. Pathohistological examination showed spindle-shaped and stellate cells which formed clusters and contained large amounts of acid polysaccharides as demonstrated by the alcian blue method.(ABSTRACT TRUNCATED AT 250 WORDS)
No To Shinkei 1986 Sep
PMID:[Cardiac myxoma metastatic to the brain]. 379 Mar 67

Traumatic hemorrhages in the basal ganglia were seen in five children. The CT findings and the characteristics of these injuries were discussed. All of children were injured in car accidents. They ranged in age from 3 to 13 years. The patients comprised 3 boys and 2 girls. On admission, case 1 was conscious and case 2 was in a stupor. Case 1 and 2 were mild cases in which spotty hematomas were seen in the ganglionic region. They recovered fully. Case 3-5 were severe cases in which massive hematomas were seen in the ganglionic region. On admission, their consciousness ranged from stupor to coma. We performed operations on two of them but their recoveries were unsatisfactory. Their results were as follows. One had residual hemiparesis and dysarthria, one experienced tetraparesis and dysarthria and the last went from coma to a vegetative state. Concerning the directions of the impacts, 4 were hit in the frontal region or face and the last one received blows on the occipital region. Skull fractures were seen in two cases. Mandibula and clavicula fractures were seen in the other two cases. Only one child had no fractures. We conclude that pediatric traumatic hemorrhages in the basal ganglia are induced by severe impact on the frontal or occipital regions. The size of hematomas in this injury is spotty or massive. The massive type has a poor prognosis. On the other hand, the spotty type's prognosis is good. We speculate that impact to the head causes a shear strain in the ganglionic region. At that time, vessels in the area are injured.
No Shinkei Geka 1985 Sep
PMID:[Traumatic hemorrhage in the basal ganglia in the child. Five cases]. 406 15


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