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)

The cerebellum's role in cognitive skills was examined in a child (L.C.) with focal injury to the left cerebellum. Initial symptoms included aphasia and dysarthria. At 3 and 9 months post-injury, clinical neuropsychological tests revealed persistent psychomotor slowing as well as deficits in executive functions. Further cognitive testing at 13 and 16 months post-injury demonstrated that L.C. processed information from both the linguistic and nonlinguistic domains more slowly than age-, grade- and sex-matched controls. Notably, her linguistic processing was more than twice as slow as that of her peers, whereas her nonlinguistic processing was only approximately 20% slower. Within each domain the degree of cognitive slowing was approximately the same across diverse tasks. These results are consistent with the hypothesis of a cerebellar contribution to cognitive processing, particularly the processing of linguistic information.
J Int Neuropsychol Soc 1998 Sep
PMID:Cerebellar contribution to linguistic processing efficiency revealed by focal damage. 974 38

A 5-year-old boy with hydrocephalus and a lumbar myelomeningocele underwent extension of peritoneal tube of ventriculoperitoneal (VP) shunt system. Prior to the operation he had been able to walk independently with the use of braces, to speak complex sentences and to sing songs. After the surgery, he lost consciousness and became critically ill with irregular respiration. He was artificially ventilated for 10 days, and then recovered, with sequelae of right facial paresis and slight dysarthria. T1 weighted magnetic resonance image showed high intensities of the medial part of the inferior lobe of the cerebellum and medulla oblongata, which were interpreted as representing edema. This case illustrates that Chiari II malformation can become symptomatic after a VP shunt trouble.
No To Hattatsu 1998 Sep
PMID:[Manifestation of Chiari II symptoms following peritoneal shunt tube extension]. 978 Jul 41

We report an 11-year-old boy with a non-photosensitive epileptic self-induced seizures, pacygyria and familial ataxia. His grandmother and aunts had dysarthria, and his mother had developed progressive ataxia and myoclonus since 40 years old. His older sister had ataxia, mental retardation and epilepsy. As for the boy, motor developmental delay with muscle hypertonicity of left extremities was recognized at the age of 5 months. Mental retardation and ataxia was recognized at the age of 3 years and slight mental regression is recognized at the age of 11 years. No special findings were detected in an examination of his blood and cerebrospinal fluid, including amino acids, lysosomal enzymes activity and genetic analysis for dentatorubralpallidoluysian atrophy. Brain magnetic resonance imaging revealed pachygyria of the right cerebral cortecies. At the age of two, he began to induce seizures with impairment of consciousness in himself by waving his right hand over his face which was directed toward a source of bright light. At the age of seven, he developed spontaneous seizures with impairment of consciousness. An EEG showed frequent spikes in the occipital areas, on the right and left sides occurring either independently or synchronously. Intermittent photic stimulation and pattern stimulation did not induce a paroxysmal discharge in EEG. Ictal EEG suggested that the origin of the seizures was the occipital lobe. Treatment with valporate and zonisamide was effective in reducing the seizures. The findings of our case imply the pathogenesis of self-induced seizures and the relationship between PME and neuronal migration disorders.
No To Hattatsu 1998 Sep
PMID:[A case of non-photosensitive, self-induced epileptic seizures with pacygyria]. 978 Jul 45

Female monozygotic twins developed upper and lower limb neurogenic weakness in their thirties, followed by cerebellar ataxia, dysarthria and death after an illness duration of about 20 years. Autopsy revealed pathological features typical of neuronal intranuclear inclusion disease (NIID) and positive ubiquitin immunostaining of the inclusions. Two adult sons of one of the twins have now developed an identical illness. This family provides strong evidence of an hereditary form of NIID.
J Neurol Sci 1998 Sep 18
PMID:Familial neuronal intranuclear inclusion disease with ubiquitin positive inclusions. 980 14

A 30-year-old female became comatose due to embolic occlusion of the basilar artery, caused by surgical injury to the origin of the vertebral artery during removal of a neurinoma in the upper thoracic paravertebral region. The basilar artery occlusion was treated by local fibrinolysis through a microcatheter. Two weeks later she recovered her consciousness but suffered mutism. Her speech disturbance was characterized by severe ataxic dysarthria known as "cerebellar mutism" but without cranial nerve paresis. The mutism gradually improved during the following 3 months. This is case of cerebellar mutism was apparently due to ischemic stroke. Disturbance by hypoperfusion of the cerebellum and brain stem may have been involved in the pathogenesis of cerebellar mutism.
Neurol Med Chir (Tokyo) 1998 Sep
PMID:Cerebellar mutism after basilar artery occlusion--case report. 980 3

Cerebellar mutism after surgery for posterior fossa tumours in children is a well-described, though rare, entity. Most of these tumours are located in the region of the cerebellar vermis extending to the hemispheres. The authors report a case of cerebellar mutism in an 8-year-old boy who underwent surgical evacuation of a spontaneous vermian haematoma. We feel that his mutism was an extreme form of cerebellar dysarthria.
Childs Nerv Syst 1998 Sep
PMID:Transient cerebellar mutism after evacuation of a spontaneous vermian haematoma. 980 55

Medial medullary infarct is a rare type of brain stem infarction first described in 1908. It was only since the broad use of MRI that an accurate clinical topographical correlation could be documented in survivors. We observed two patients; one of them had an anteromedial unilateral infarction in the upper medulla, and the clinical picture was characterized by contralateral hemiparesis, facial weakness, dysarthria and palatal weakness. The outcome was good. The second patient however showed a severe tetraparesis with only minor brain stem signs, secondary to bilateral anteromedian infarction with a large craniocaudal extension. He survived with severe residual impairment. The most likely cause of infarction was arteriosclerosis of the A. vertebralis and A. spinalis anterior, respectively. These two different cases suggest that the medial medullary syndrome is heterogeneous comprising small unilateral infarctions with mild clinical signs as well as large bilateral infarctions with a poor outcome.
Wien Klin Wochenschr 1998 Sep 18
PMID:[Medial medullary infarct]. 981 32

The 1960s were a period of great flowering in the recognition of neurologic disorders in children. The so-called ataxic cerebral palsies were an especially fertile field waiting for clarification. Congenital ataxia coupled with hyperpnea-apnea, abnormal eye movements, and retardation was identified as an autosomal-recessive syndrome eponimically associated with the senior author, Marie Joubert. The disorder, though rare, is increasingly recognized and a lay society dedicated to family support and research has been formed. In preparation for a recent symposium the original proband was re-examined 30 years later and the manifestations in adults clarified. Severe dysarthria was the most striking feature in this man, the hyperpnea-apnea had diminished, and the abnormal eye movements were less striking. Ataxia was still present but not severe. Poor judgment and borderline intelligence rounded out the clinical picture. Modern imaging has clarified, in part, the anatomic basis of this syndrome.
J Child Neurol 1999 Sep
PMID:History of Joubert syndrome and a 30-year follow-up of the original proband. 1048

The patient was a 72-year-old man who had a history of subtotal gastrectomy for gastric ulcer at age of 37 years. He had no familial history of hereditary disorders. In 1980 he noticed mild ataxic gait which exaggerated while he closed eyes. The symptoms increased gradually, and four years later he noticed hypoesthesia of his soles. In 1983 he was admitted to the National Center Hospital for Mental, Nervous and Muscular Disorders for the first time. Neurological examination revealed dysarthria, ataxic gait, disturbance of coordination to a slight degree, and muscle strength of the upper and lower limbs were in normal range. Mild hypoesthesia of pain and temperature sensation, and marked decrease of deep sensation and vibration of the lower extremities were demonstrated. Romberg sign was positive. EMG studies revealed low amplitude of action potential and normal motor nerve conduction velocity. Biopsy of the sural nerve showed marked decrease of both large and small myelinated fibers. In 1998 he was admitted second time for the further examination. Laboratory examination including routine blood examination, blood chemistry including CRP, TPHA, vitamin B1, B2, B12, A, E, K, hexosaminidase A in leucocyte were in normal range. CSF was normal. Genetic studies including SCA 1, 2, 3, 6, DRPLA, CMT1A, CMTX 1 were all negative. MCV of lower limbs was in normal range, though SCV was not evoked in the upper and lower limbs. MRI studies showed mild atrophy of the bilateral lobulus of the cerebellum which was not so much changed in the last 5 years. The clinical symptoms revealed dominant posterior column disturbance, ataxia and sensory neuropathy. These combination was not described in the previous literature, and this case may be a new variant of the spinocerebellar degeneration.
Rinsho Shinkeigaku 1999 Sep
PMID:[A case with posterior column ataxia associated with cerebellar ataxia and sensory neuropathy]. 1061 59

We describe a case of unilateral IX, X and XI cranial and upper cervical nerve palsies involving zoster sine herpete (ZSH). A 63-year-old man experienced nausea, loss of appetite and general fatigue. On 4 days of illness, dysphagia, dysarthria and difficulty in elevation of his right arm appeared. Neurological examination showed the right curtain sign, a nasal voice and a decreased right gag reflex. He could hardly elevate his right arm laterally. Needle electromyography revealed positive sharp waves in his right trapezius muscle. Although no skin lesion was detected, anti-varicella-zoster virus antibodies were positive in both serum and cerebrospinal fluid. Acyclovir and a steroid were ineffective for these symptoms. Although case reports of unilateral IX, X and XI cranial nerve palsies with ZSH is very rare, ZSH should be kept in mind in the differential diagnosis of multiple cranial nerve palsies.
Rinsho Shinkeigaku 1999 Sep
PMID:[A case of zoster sine herpete with involvement of the unilateral IX, X and XI cranial and upper cervical nerves]. 1061 62


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