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)

Transient mutism resolving to cerebellar speech after posterior fossa surgery is a well-recognized phenomenon, particularly in pediatric patients. The anatomic basis for this postoperative functional change is unclear but may reside in the dominant superior cerebellar hemisphere or the medial deep cerebellar nuclei. We report a case of an 8-year-old girl who presented for surgical resection of a cavernous malformation of the right pons (at the level of the middle cerebellar peduncle) after hemorrhage. Preoperatively, her complaints consisted of contralateral motor deficits. She had normal speech. Her lesion was resected through a subtemporal approach to the pons. She awoke unable to speak. She was able to communicate through a variety of verbal cues, including sign language. Her mutism lasted 12 days after which she underwent a prolonged period of slowly resolving cerebellar dysarthria. Her preoperative motor deficits also slowly resolved. This is the first reported case of mutism resolving to cerebellar dysarthria after a supratentorial approach to the brain stem. We discuss the anatomic basis for postoperative mutism in light of previous observations combined with the unusual finding of mutism after pontine surgery. In particular, reports of mutism after bilateral cerebellar hemispheric injury, bilateral or unilateral medial deep nuclear injury, and, now, pontine tegmental injury implicate the superior cerebellar hemispheres, the deep cerebellar nuclei, and the nuclear outflow through the superior cerebellar peduncle as the anatomic bases for cerebellar participation in the production of human speech.
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PMID:Mutism and cerebellar dysarthria after brain stem surgery: case report. 759 21

Alteration of speech is a rare but distressing complication of orthotopic liver transplantation (OLT). We describe a characteristic speech disorder identified in a large series of consecutive patients undergoing OLT. Between 1988 and 1993, 525 adults underwent OLT. For all recipients with neurologic complications, we reviewed clinical findings, imaging and electrophysiologic test results, and perioperative laboratory data. Five patients (ages 23-52; UNOS status 3-4) exhibited a characteristic pattern of stuttering dysarthria, leading to complete loss of speech production, occasionally with elements of aphasia. In four of the five patients, right-sided focal seizures were subsequently noted. All cases presented within the first 10 postoperative days and improved with 1 month of cessation of cyclosporin (CyA), although halting, monotonous speech was evident to some degree in all five for up to 1 year. There was no correlation between onset of symptoms and CyA levels. None of the patients has clinical or radiologic findings suggestive of central pontine myelinolysis or akinetic mutism. EEGs and Spect scan results were consistent with dysfunction in the left frontotemporoparietal regions of the brain. A characteristic speech disorder, which may be described as cortical dysarthria or speech apraxia, occurs in approximately 1% of adults undergoing OLT. Prompt recognition of this syndrome and temporary cessation of CyA therapy may favorable affect the course.
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PMID:Loss of speech after orthotopic liver transplantation. 762 86

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism. The corresponding gene locus has been localized on the long arm of chromosome 13. Three different clinical variants of the disease can be distinguished: hepato-cerebral, abdominal/hepatic, and central nervous type. The heterogeneity of symptoms can cause problems in differential diagnosis, especially when another concordant disorder can also explain the pathogenesis of symptoms. The case report of a young man who suffered from brainstem contusion demonstrates the possibilities of misinterpretation because presenting symptoms could be attributed either to traumatic brain injury followed by adjustment disorder or Wilson's disease. Clinical signs included leftsided hemiparesis, bilateral gaze direction nystagmus, marked dysarthria with consecutive pervasive mutism, choreo-athetoid movements, spasmodic torticollis and diplopia dependent on gaze direction. Slit lamp examination showed Kayser-Fleischer's corneal ring. EEG- and computer assisted tomography investigations revealed non-specific findings. The patient was treated with D-Penicillamine. Alternative treatment with oral zinc preparations is discussed.
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PMID:Case report: concordant traumatic brainstem contusion delayed diagnosis in a young man with Wilson's disease. 778 83

A 40-year-old male patient is presented who survived Marchiafava-Bignami disease and recovered sufficiently to be assessed neurologically and neuropsychologically in some detail. Besides dementia, lack of initiative, and psychomotor retardation here ascribed to extracallosal damage, he showed a number of symptoms of hemispheric disconnection such as left-sided apraxia, poor bimanual coordination in specific laboratory tests, and deficits in the interhemispheric transfer of somaesthetic information. Other commissural functions, such as interhemispheric transfer of tactile, visual and auditory information as well as bilateral coordination in previously overlearned tasks, were nearly intact. The observed dysarthria could be meaningfully discussed in relationship to postcallosotomy mutism. It is concluded, that the partial interhemispheric disconnection syndrome in Marchiafava-Bignami disease lacks functional compensation which is different from the usual course in partial commissural section.
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PMID:Incomplete split-brain syndrome in a patient with chronic Marchiafava-Bignami disease. 784 Aug 88

An 8-year-old girl developed mutism after removal of a vermian medulloblastoma. The mutism was not accompanied by long tract signs or cranial nerve palsy. The girl started to regain her speech 2 weeks postoperatively, showing marked improvement 2 months after the operation, after passing through a dysarthric phase. Magnetic resonance imaging showed only normal postoperative changes without abnormalities of dentate nuclei or supranuclear region. Nineteen similar cases of transient mutism following cerebellar operations have been reported in the literature, most of them with a delayed postoperative onset. In all patients the recovery of speech started to appear 4 days to 4 months postoperatively, and all patients passed through a monotonous, dysarthric phase. The absence of long tract or other brain stem signs, together with the presence of dysarthria during the recovery of speech, suggested a cerebellar cause of the transient mutism. Various hypotheses advanced to explain the pathogenesis of this speech disorder are analyzed.
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PMID:Transient mutism after posterior fossa surgery. 792 41

Mutism can be defined as "a condition in which there is no, or very little, oral-verbal expression, whereas comprehension of speech ... is normal or at least at a considerably higher level" (Lebrun, 1990). Benson (1979) enumerates five neuroanatomical correlates of mutism: (a) damage to the Broca region, (b) lesion of the supplementary motor area of the dominant hemisphere, (c) dysfunction of the mesencephalic reticular system, (d) thalamotomy, and (e) bilateral pathology of cortical and subcortical motor structures. The last item refers to syndromes of mutism resulting from central motor disorders. Depending on location and size of the lesion this pathophysiological interpretation should hold true for the fourth point as well. In mutism due to central motor disturbances lacking verbal expression represents the most severe degree of dysarthria, i.e. anarthria. The present review provides a detailed description of mutism following corticobulbar, striatal, and cerebellar dysfunctions.
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PMID:[Mutism in central motor disorders--a review of the literature]. 795 17

"Cerebellar" mutism refers to a specific childhood disorder in which a complete but transient loss of speech, followed by dysarthria, occurs after removal of a cerebellar tumor. We present a consecutive series of 15 children with this disorder, which we prefer to designate "mutism and subsequent dysarthria." The conditions in which it develops suggest also an extracerebellar component of cerebellar mutism. Hydrocephalus at presentation, localization of tumor adjacent to the fourth ventricle, and postsurgical edema of the pontine tegmentum are involved in its development.
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PMID:The syndrome of 'cerebellar' mutism and subsequent dysarthria. 796 50

Language disturbances in cerebral palsy with mutism. The analysis of the afferent side of language in 18 cerebral palsy children with no oral expression shows that they form a very heterogeneous population. The semiological analysis concerning comprehension leads to their classification into four main groups akin to the classical dysphasia syndromes. Major agrammatism occurring in phonologico-syntactic dysphasia. Sensory dysphasias (or verbal deafness). Mixed (or global) dysphasias. Motor dysphasias: phonological programmation disturbances (anarthria) and motor production disturbances (dysarthria). The disclosure of these various dysphasias allows for specific rehabilitation and for education procedures suitable for each individual patient.
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PMID:[Symptomatology of language disorders in cerebral palsy with mutism]. 821 52

MUTISM AND A variety of other neurobehavioral symptoms have been reported anecdotally after the removal of posterior fossa mass lesions. To determine the incidence and clinical spectrum of this syndrome, a detailed review was performed of patients undergoing resection of infratentorial tumors at our institution during the last 9 years; 12 of 142 patients (8.5%) manifested this syndrome, the largest series of such patients reported to date. Each child had a lesion that involved the vermis; seven had medulloblastomas, three had astrocytomas, and two had ependymomas. The incidence among children with vermian neoplasms was 13%. Ten children underwent division of the inferior vermis during tumor resection, and three had a superior vermian incision; one child underwent both superior and inferior vermian incisions. In 10 children, mutism developed in a delayed fashion postoperatively. The speech disturbance was associated with poor oral intake in 9 children, urinary retention in 5, long-tract signs in 6, and bizarre personality changes, emotional lability, and/or decreased initiation of voluntary movements in all 12. Neuropsychiatric testing, performed in seven children, confirmed impairments not only in speech but also in initiation of other motor activities. Ten children regained normal speech, bladder control, and neurological functioning, other than ataxia and mild dysarthria, within 1 to 16 weeks; two children had significant residual deficits. Characteristically, affect and oral intake returned to their preoperative baseline before the speech difficulties began to resolve. A detailed radiological review of these cases in parallel with 24 cases of vermian tumors without mutism identified only one factor that was significantly associated with the mutism syndrome, bilateral edema within the brachium pontis (P < 0.01). Neither the size of the tumor nor the length of vermian incision was associated with the development of mutism. The clinical features of this syndrome in the context of these imaging findings suggest that the mutism syndrome results from transient impairment of the afferent and/or efferent pathways of the dendate nuclei that are involved in initiating complex volitional movements. The clinical courses of our patients are presented and compared with those of similar cases in the literature in an attempt to evaluate the validity of this hypothesis.
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PMID:Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. 872 37

Cerebellar mutism has been reported after surgery for posterior fossa tumors in children and, rarely, in adults. The pathogenesis of this syndrome remains unclear, and controversy exists regarding whether it is a purely psychogenic disorder or an organic syndrome. The anatomical substrate for the mutism also remains unknown. We encountered five cases of postoperative transient cerebellar mutism in a consecutive series of 63 children with posterior fossa tumors. These cases were analyzed and compared with the patients without mutism to find predictive factors for the occurrence of mutism, with the hope of elucidating further the pathophysiological mechanism. The most significant finding was the presence in all cases of a period of cerebellar dysarthria after resolution of the muteness. We, therefore, believe that cerebellar mutism is an extreme form of dysarthria, rather than a real cognitive deficit or a psychological disturbance.
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PMID:Transient cerebellar mutism after posterior fossa surgery in children. 872 37


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