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PubMed2004

Akinetic mutism following stroke.

This is an appraisal of the varied clinical presentation and the neural substrate for akinetic mutism following stroke. The diagnosis is important as akinetic mutism is often misdiagnosed as depression, delirium and locked-in-syndrome. This is a descriptive study of eight selected patients with akinetic mutism following infarction/haemorrhage in different regions of the brain with characteristic syndromes. They involved the critical areas namely, the frontal (cingulate gyrus, supplementary motor area and dorso-lateral border zone), basal ganglia (caudate, putamen), the mesencephalon and thalamus. The disorders of speech and communication took different forms. The speech disorder included verbal inertia, hypophonia, perseveration, softened and at times slurred. The linguistic disturbances were fluent, non-fluent, anomia and transcortical (motor, mixed) aphasias. The findings were related to what is known about the neuroanatomic location of the lesions and the role of the frontal-subcortical circuitry in relation to behaviour. Akinetic mutism could be explained by damage to the frontal lobe and or interruption of the complex frontal subcortical circuits.

Adult↗
PubMed1999

A double-blind comparison of lorazepam and oxazepam in psychomotor retardation and mutism.

BACKGROUND: An increasing number of case reports indicate a superior therapeutic response of catatonialike symptoms, such as severe psychomotor disturbance and mutism, associated with psychiatric disorder to the benzodiazepine lorazepam (LO). Equivocal results, however, are also reported with regard to other benzodiazepines for the treatment of this syndrome. The purpose of this study was to compare the effects of LO and oxazepam (OX), benzodiazepines with comparable pharmacokinetics, on psychomotor retardation and mutism associated with psychiatric disorder. METHODS: Twenty-one hospitalized patients with severe psychomotor retardation and mutism were treated with 2 mg LO and 60 mg OX in a double-blind crossover study design. RESULTS: Both benzodiazepines significantly reduced psychomotor symptoms. When administered for the first time, 4 of 7 patients with LO and 6 of 10 patients with OX improved at least 50% on visual analog scale (VAS) rating. Reduction in symptoms was significant with LO and OX treatment on either day of treatment. The second time, however, LO was significantly better compared with OX in alleviating the target symptoms. CONCLUSIONS: Both OX and LO are effective for the treatment of psychomotor retardation. Thus, the beneficial effect of LO on psychomotor retardation and mutism is not a unique pharmacodynamic property but more likely due to its pharmacokinetic profile. The differential effect of the two benzodiazepines on the second day of treatment warrants further clarification. Several hypotheses are evaluated.

Adult↗
PubMed1998

Post-traumatic mutism in children: clinical characteristics, pattern of recovery and clinicopathological correlations.

Among the numerous clinical syndromes observed after severe traumatic head injury, post-traumatic mutism is a disorder rarely reported in adults and not studied in any detail in children. We report seven children between the ages of 3 1/2 and 14 years who sustained severe head injury and developed post-traumatic mutism. We aim to give a precise clinical characterization of this disorder, discuss differential diagnosis and correlations with brain imaging and suggest its probable neurological substrate. After a coma lasting from 5 to 25 days, the seven patients who suffered from post-traumatic mutism went through a period of total absence of verbal production lasting from 5 to 94 days, associated with the recovery of non-verbal communication skills and emotional vocalization. During the first days after the recovery of speech, all patients were able to produce correct small sentences with a hypophonic and monotonous voice, moderate dysarthria, word finding difficulties but no signs of aphasia, and preserved oral comprehension. The neurological signs in the acute phase (III nerve paresis in three of seven patients, signs of autonomic dysfunctions in five of seven patients), the results of the brain imaging and the experimental animal data all suggest the involvement of mesencephalic structures as playing a key role in the aetiology of post-traumatic mutism.

Adolescent↗
PubMed2006

Pure mutism due to simultaneous bilateral lenticulostriate artery territory infarction.

A case of pure mutism without pseudobulbar palsy and other neurological findings resulting from simultaneous bilateral lenticulostriate artery territory infarction is presented. A 45-year-old woman suffered a transient ischemic attack with nonfluent aphasia and right hemiparesis. Six months later, she developed pure mutism without oral apraxia, pseudobulbar signs, and motor deficits. Magnetic resonance imaging revealed bilateral infarction in the lentiform nucleus regions. In the available data, there is only one report of simultaneous bilateral lenticulostriate infarction. To date, in all reported cases of mutism of subcortical vascular origin there are also various degrees of pseudobulbar signs and motor deficits and the responsible lesions are mostly consecutive. The case presented here is the first to show pure vascular mutism without other neurological findings.

Acute Disease↗
PubMedDate not supplied

Treating selective mutism in a paediatric rehabilitation patient by altering environmental reinforcement contingencies.

Selective mutism is a disorder which can cause severe social and academic impairment, and for which a wide variety of treatment approaches have been used, with varying degrees of success. Selective mutism can be conceptualized as the lack of generalization of a class of operant responses (e.g. audible and comprehensible verbalizations) across environmental contexts. The rehabilitation hospital setting, in which the patient is seen daily by multiple people in multiple settings, is particularly well-suited for implementing a systematic behavioural intervention to establish verbal behaviour and simultaneously reinforce its generalization. Data are presented on a 7-year-old female admitted to a rehabilitation hospital following orthopaedic surgery, who met the DSM-IV diagnostic criterion for selective mutism. Additional medical diagnoses included cerebral palsy, microcephaly, and mild mental retardation. A behavioural programme was developed and implemented to reinforce differentially first any communication, then verbal communication across staff and settings. Results were evaluated using a modified multiple baseline across settings design, and demonstrate that verbal, written, and tangible reinforcement effectively increased verbal behaviour where it previously rarely occurred. Results are discussed in terms of the relationship between selective mutism, social phobia and related disorders. The theoretical roles of behavioural phenomena (discriminative stimuli, stimulus generalization) in the development and treatment of these disorders are discussed.

Behavior Therapy↗
PubMed1997

Systematic assessment of 50 children with selective mutism.

OBJECTIVE: To clarify the diagnostic significance of selective mutism (elective mutism in DSM-III-R). METHOD: Fifty children with selective mutism were evaluated systematically by means of semistructured clinical interviews and rating scales to obtain detailed diagnostic information. RESULTS: All 50 children met DSM-III-R criteria for social phobia or avoidant disorder and 24 (48%) had additional anxiety disorders. Clinical measures of anxiety and behavioral symptoms supported the presence of anxiety disorders as a characteristic of selectivity mute children. Only one case each of oppositional defiant disorder and attention-deficit hyperactivity disorder was found. CONCLUSIONS: Persistent selective mutism typically presents in the context of anxiety disorders.

Adolescent↗
PubMed1998

Visual impairment associated with mutism after posterior fossa surgery in children.

OBJECTIVE: To report four children with visual impairment associated with mutism after posterior fossa surgery. Mutism after posterior fossa surgery is a well-described phenomena, but to our knowledge, visual impairment has not been reported in association with it. METHODS: Record review of four children (age range, 3-7 yr) who underwent posterior fossa surgery (via suboccipital craniotomies) for removal of a medulloblastoma (three patients) or ependymoma (one patient). Each presented with headache, ataxia, or nausea and vomiting, but none had preoperative visual complaints other than diplopia. Postoperatively, all patients were mute, and because of apparent visual loss, neuro-ophthalmic consultation was requested. Postoperative scans and examinations were also reviewed. RESULTS: Each child was awake but appeared withdrawn without verbal output. No child blinked to threat or fixed or followed. In each case, pupillary reactivity was normal, and funduscopic examinations revealed only papilledema. One child reached for money. Within weeks or months postoperatively, the mutism spontaneously resolved, and visual behavior in general improved, roughly in parallel. During the follow-up period, papilledema resolved and the disc color was normal in each case. Magnetic resonance images obtained postoperatively revealed nothing remarkable, except surgical defects, without lesions in the retrogeniculate pathway. CONCLUSION: Impaired visual behavior, mimicking cortical visual loss, may be associated with mutism after posterior fossa surgery in children. The prognosis for recovery is excellent and parallels the return of normal speech. The mechanism is unclear.

Brain Neoplasms↗
PubMed1997

Selective mutism: a population-based study: a research note.

Seven-year-olds to 15-year-olds in 2 school districts of Göteborg, Sweden, were screened for selective mutism by their teachers and follow-up was achieved for a full school year. Three girls and 2 boys met DSM-IV criteria for selective mutism and a further 25 had a combination of shyness and reticence that did not amount to clinical disorder. The rate of typical selective mutism was 18 in 10,000 children. Shyness/reticence occurred in 89 in 10,000 children. Selective mutism was more common than suggested by earlier studies. Teachers of school age children need to be better informed about its existence.

Adolescent↗
PubMed1995

Mutism developing after bilateral thalamo-capsular lesions by neuro-Behçet disease.

We described a 44-year old right-handed man showing mutism, left hemiplegia and pseudobulbar palsy after CT and MRI documented bilateral thalamo-capsular lesions by neuro-Behçet disease. Single photon emission tomography (SPECT) and Xenon CT revealed hypoperfusion of the bilateral frontal lobes. The pathophysiological mechanism of mutism was discussed and we postulate that mutism might occur as the result of frontal lobe dysfunction due to the disconnection of thalamocortical fiber from thalamus to frontal cortex and that it could be interpreted as an incomplete form of akinetic mutism.

Adult↗
PubMed1990

Cyclosporin-associated akinetic mutism and extrapyramidal syndrome after liver transplantation.

Three patients developed akinetic mutism on the third day after the introduction of intravenous cyclosporin A, given for immunosuppression after liver transplantation. One patient in addition developed a florid orofacial dyskinesia, which resolved completely, as did the akinetic mutism, after withdrawal of cyclosporin. In these patients the time course of the akinetic mutism and extrapyramidal syndrome, which developed in the absence of any other identifiable cause, suggests cyclosporin A was the precipitating factor. Subsequently, two of the patients showed signs of pseudobulbar palsy with abnormalities in the pons on MRI scanning, suggesting central pontine myelinolysis (CPM). None of the patients had experienced significant fluctuations in serum sodium or other risk factors for central pontine myelinolysis and the exact relationship to the earlier cyclosporin related mutism was not clear.

Adult↗
PubMed1986

Hyperkinetic mutism: bilateral ballism and basal ganglia calcification.

We studied a 70-year-old woman with a unique combination of hyperkinesia and mutism. These findings differed from akinetic mutism because there was continuous bilateral ballism and dystonia--hence the term "hyperkinetic mutism." CT demonstrated bilateral calcifications in the basal ganglia, and MRI indicated bilateral watershed infarcts. Different dopaminergic mechanisms may underlie the hyperkinesia and mutism.

Aged↗
PubMedDate not supplied

Linguistic and cognitive deficits associated with cerebellar mutism.

Cerebellar mutism is a period of transient loss of speech following posterior fossa surgery, usually in children under 15 years of age. To date, cases presented in the literature have addressed primarily the motor speech aspect of the phenomenon, with little documentation relative to higher-level linguistic and cognitive functioning of these children. Recent research, however, is increasingly documenting evidence of cerebellar involvement in language and cognition. This paper describes four children, three of whom presented with a period of mutism following posterior fossa surgery, and one who did not exhibit mutism. All children, however, demonstrated a similar profile of significant high-level linguistic and cognitive deficit on formal speech-language and neuropsychological measures. Documented cases to date are reviewed and compared to these subjects. Literature documenting the role of the cerebellum in language and cognition is summarized and compared to evidence of residual deficits associated with cerebellar mutism as demonstrated by the children in this study.

Adolescent↗
PubMed1983

Mutism as a consequence of callosotomy.

Transient mutism has been reported following commissurotomy and callosotomy. The cause for this mutism is unknown. A case of mutism following callosotomy is presented, and the preoperative and postoperative data on neurological, physiological, and psychological functioning are discussed. The data suggest that the mutism is not caused by general intellectual deterioration, cortical lesion, or peripheral damage affecting speech production. The syndrome may result from severing interhemispheric connections in cases where both hemispheres are required for speech production.

Adult↗
PubMed2006

Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children's Oncology Group.

OBJECT: Cerebellar mutism syndrome (CMS) is a unique postoperative syndrome typically arising 1 to 2 days after resection of a midline posterior fossa tumor; it consists of diminished speech progressing to mutism, emotional lability, hypotonia, and ataxia. Most descriptions have been limited to small institutional series using a retrospective chart review methodology. METHODS: The authors incorporated a CMS questionnaire in two large clinical trials (Children's Cancer Group [CCG] 9931, treatment for high-risk medulloblastoma/primitive neuroectodermal tumor; and CCG/Pediatric Oncology Group [POG] A9961, treatment for average-risk medulloblastoma) to prospectively survey for incidence, severity, and possible causes of CMS in children with newly diagnosed medulloblastoma. Information pertaining to 450 of the 463 patients enrolled in the studies was available for review (82 patients in CCG 9931, and 368 patients in CCG/POG A9961). Cerebellar mutism syndrome occurred in 107 (24%) of 450 children. Symptom intensity was judged to have been severe in 43%, moderate in 49%, and mild in 8% of these 107 patients. Mutism and ataxia were the features most frequently judged as severe. In both cohorts, preoperative brainstem invasion was the only feature that correlated with risk of CMS. One year after diagnosis, nonmotor speech/language deficits, neurocognitive deficits, and/or ataxia persisted in a significant fraction of patients. CONCLUSIONS: Nearly one quarter of patients who underwent resection of a medulloblastoma developed symptoms of CMS, of which 92% were judged to be of moderate or severe intensity. Brainstem invasion by tumor was the only risk factor that correlated positively with CMS occurrence; there was a negative correlation with cerebellar hemisphere tumor location. As more radical resections are attempted for medulloblastoma, the potential for increased morbidity must be carefully weighed against prognostic factors, especially in patients with brainstem invasion.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1975

[Akinetic mutism and bicingular softening. 3 anatomo-clinical cases].

The authors describe three pathological cases of akinetic mutism with, as a common basic lesion, bilateral infarction of the cingulate gyrus secondary to aneurysm of the anterior communicating artery (case n degrees 1), to a huge olfactory meningioma (case n degrees 2), both operated on, and to atheromatous occlusion of the anterior cerebral arterial system (case n degrees 3). These three cases enable a variety of "anterior and waking" akinetic mutism to be described which is unusual enough to be compared with other mesencephalic and diencephalic aspects of this syndrome. It is in fact an akinetic mutism characterized by: a certain dissociation in its non-response to various stimuli, a particularly marked appearance of wakefulness when day-time alertness is considered, conservation of the waking-sleeping rhythm, perception and reaction unpredictable and paradoxical in both degree and quality, complete absence of any spontaneous verbal communication in contrast to relative break-down of solicited communication which is infrequent, uncertain and unresponsive to the usual methods of stimulation, without any possibility of a code. In addition, there is a remarkable mimic and segmental general akinesia, resistant to the usual nociceptive stimuli, but sensitive to slight excitation of the manual and oral zones. Besides this special akinetic mutism, there are variously systematised signs, mostly asymmetrical, indicating lesion of the cortico-sub-cortical frontal structures bordering on the gyrus cinguli. This unusual behaviour pattern corresponds in these three cases to extensive anterior bilateral ischemic lesions of the cingulate gyrus regularly associated with bilateral infarctions confined to the medial aspect of F1 in the superficial territory of the two anterior cerebral arteries, to possible neurosurgical changes (ablation of the right frontal pole) and to compressive or ischaemic lesions of the gyrus rectus. These exclusively cortico-sub-cortical associated lesions are in contrast with the remarkably intact caudate nuclei, the pallidal, thalamic, hypothalamic and septal formations and the anterior pillars of the fornix. These findings compared with the results of experimental research carried out by M. Kennard, help, if help is needed, to resolve the apparent contradictions between the effects of therapeutic cingulectomies or cingulotomies and the scanty pathological data already available in cerebral vascular pathology.

Adult↗
PubMed2001

[An adult case of cerebellar mutism after removal of cerebellar hematoma].

Cerebellar mutism is a well documented complication of posterior fossa tumor surgery in children. But only one case presenting with cerebellar mutism after removal of a cerebellar hematoma in adults was reported previously. We present 64-year-old male developing cerebellar mustism after surgery for hypertensive cerebellar hemorrhage. A cerebellar hematoma was located in the right side dentate nucleus and cerebellar peduncle dominantly not extending to the cerebellar vermis. During the period of cerebellar mutism, single photon emission computed tomography revealed a decrease in the contralateral temporo-parietal cerebral blood flow. Cerebellar mutism in this case may be associated with the low blood flow in the contralateral supplementary motor cortex owing to injury to the dentate-thalamo-cortical pathway at the dentate nucleus and cerebellar peduncle. The relevant literature is reviewed.

Cerebellar Diseases↗
PubMedDate not supplied

[Postoperative mutism in children with the posterior fossa tumors].

Over 10 years (1990-1999), the Pediatric Department, Ukrainian Institute of Neurosurgery named after Academician A. P. Romodanov, operated on 376 children with posterior fossa tumors. Thirty two 8.7% children developed a posterior fossa syndrome and cerebellar mutism syndrome postoperatively. This paper gives a detailed analysis of the date available in the world literature on the incidence and causes of mutism, and mechanisms of its development. Many factors predisposing to mutism were identified, but no anatomic structures responsible for its formation were found. In the authors' opinion, mutism may not result from anatomic structures, but from the functioning of afferent or efferent connections to this brain region bilaterally.

Adolescent↗
PubMed2003

[Elective mutism--a disorder of social functioning or an emotional disorder?].

Elective mutism is a relatively uncommon disorder characterized by consistent failure to speak in specific social settings, such as in school, despite speaking fluently in a variety of other situations. Elective mutism is a very debilitating disorder for the affected child, as well as for their families, with detrimental implications for the development of social skills and learning if not offered appropriate support and treatment. The most promising treatment results have thus by far been achieved through a combination of cognitive behaviour therapy and supportive cooperation between parents, the school, and the treating child psychiatrist, occasionally supplemented with pharmacological treatments such as selective serotonin reuptake inhibitors. The available literature on this disorder is still very limited and consists predominantly of case reports. In recent years, however, there has been a growing focus on the etiology and general understanding of the disorder. While still disputed, it has been suggested that elective mutism might be understood as an emotional disorder on a par with e.g. separation anxiety and social phobia. Studies looking at the long-term course of the disorder are urgently required to elucidate whether children presenting elective mutism will be more likely to suffer from emotional disorders in adulthood, and may also ultimately lead to improved treatments for this disabling psychiatric disorder of childhood.

Adult↗