Akinetic mutism and mixed transcortical aphasia following left thalamo-mesencephalic infarction

A 54-year-old man developed somnolent akinetic mutism and acute mixed transcortical aphasia following a left thalamo-mesencephalic infarction. He also exhibited behavioural changes, namely apathy, slowness, lack of spontaneity, disinhibition, perseveration, gait apraxia and incontinence consistent with frontal lobe dysfunction. Presumably the akinetic mutism and language dysfunction were due to the thalamic stroke. All the manifestations could be related to interruption of the frontal-subcortical circuitry.     

Development of akinetic mutism and hyperphagia after left thalamic and right hypothalamic lesions

A case of childhood post-traumatic akinetic mutism is presented. The patient showed a hyperphagic condition while recovering from akinetic mutism. He had lesions in the left interlaminal nucleus of the thalamus, right globus pallidus, and right dorsomedial nucleus of the hypothalamus. Laboratory data indicated slightly disturbed hypothalamic functions. In general, akinetic mutism can be seen with bilateral destructive lesions, while hyperphagia may occur after destruction of dorsomedial hypothalamic nucleus, but it is very rare. This is the first reported case of akinetic mutism caused by a unilateral lesion.     

BIPLEDs in akinetic mutism caused by bilateral anterior cerebral artery infarction.

INTRODUCTION: Akinetic mutism is described as a result of many disorders. Bilateral infarction of the anterior cerebral artery (ACA) territory is reported rarely, however, often leading to akinetic mutism. CASE REPORT: We report a 70 year-old man with akinetic mutism due to bilateral ACA infarction. Electroencephalography, 24h after admission, showed bilateral independent periodic lateralized epileptiform discharges (BIPLEDs) in the frontal region and diffuse theta and polymorphic delta activity. DISCUSSION: Postanoxic encephalopathy, central nervous system infection and chronic seizure disorders are the major causes of BIPLEDs. However, BIPLEDs may occur in bilateral ACA territory infarction.     

Full recovery of Akinetic Mutism after corpus callosum infarction in post subarachnoid hemorrhage of ruptured distal anterior cerebral artery aneurysm

The authors report a previously healthy 23-year-old male patient who presented with subarachnoid hemorrhage and was found to have a ruptured right distal anterior cerebral artery aneurysm. He was treated by endovascular coiling technique, which was uneventful perioperatively. After a few days of mechanical ventilation and upon extubation, he expressed symptoms of apathy, drowsiness, lack of motivation, and lack of spontaneous motor function consistent with akinetic mutism. The magnetic resonance imaging of the brain revealed infarction of the whole body of corpus callosum. He remained in akinetic mutism status for twenty-one days before he started to show improvement until he fully recovered in 3 months. The authors report a unique finding where akinetic mutism resulted from infarction of the corpus callosum rather than medial frontal lobe (cingulate gyrus).

Akinetic mutism (AM) is a syndrome of psychiatric, verbal, and motor components. It is a state of limited responsiveness to the environment in the absence of gross alteration of sensorimotor mechanisms operating at a more peripheral level. Neither paralysis nor coma accounts for the symptoms.1,2 The AM may result from trauma, infarctions, infections or others. AM have been rarely reported as a consequence of aneurysmal subarachnoid hemorrhage. The authors report a case of young male patient who is previously healthy, who presented with aneurysmal SAH. He developed AM as a result of corpus callosum infarction without involvement of cingulate gyrus. The novel finding in our study is that infarction did not affect the cingulate gyrus nor medial frontal lobes. In fact, the insult was limited to the body of corpus callosum only, sparing both the genu and the splenium.     

Cerebellar lesions in the PICA but not SCA territory impair cognition

The authors sought to clarify whether lesions in different parts of the cerebellum result in differential cognitive and affective impairment. Six subjects with cerebellar lesions due to posterior inferior cerebellar artery (PICA) infarction, five subjects with lesions in the SCA vascular territory, and 11 matched controls were administered a battery of standard neuropsychological tests. PICA lesions but not SCA lesions resulted in cognitive and affective deficits pointing to a dominant role of posterior cerebellar regions in cognitive and affective processing.     

A case of dural arteriovenous malformation with bilateral thalamic infarction

A 59-year-old man was admitted because of frequent vomiting and obtundation in February 1982. Neurological examination on admission revealed only slight impairment of consciousness. Papilledema, meningeal irritation sign and paralysis were not elicited. The plain CT scan was normal, but the CT scan with contrast material showed patchy enhancement in the left temporal lobe and around the third ventricle. Cerebral angiography showed a dural arteriovenous malformation (dural AVM) in the left transverse sinus fed by the left occipital artery, and the retrograde flow into the straight sinus. By the third day following admission, the level of consciousness became alert. The patient did not complain of headache, bruit and visual disturbance. He showed mild disorientation and memory disturbance. But his ordinary daily-living was independent. In August 1982, the patient gradually became inactive and apathetic. At times he lay in bed with moving his eyes, swallowing foods. At other times, he lay in bed with closing his eyes, immobile, and unresponsive except to strong painful stimuli. The patient was incontinent and required nursing care. During three month periods, the patient progressively became somnolent, speechless and immobile. Eventually, he was in a state of akinetic mutism. The patient became unresponsive. The state of consciousness fluctuated within a narrow range. The pupils were isocoric and did not react to light. He sometimes moved his eyes horizontally, but the vertical eye movement was limited. Deep tendon reflexes were hyperactive with Babinski reflex bilaterally. Passive mobilization of extremities revealed hypertonic. The CT scan disclosed the bilateral symmetrical infarction of the thalamus.(ABSTRACT TRUNCATED AT 250 WORDS)     

Posterior Fossa Syndrome in an Adult Patient Following Surgical Evacuation of an Intracerebellar Haematoma

The posterior fossa syndrome (PFS) consists of transient cerebellar mutism, cognitive symptoms and neurobehavioural abnormalities that typically develop in children following posterior fossa tumour resection. Although PFS has been documented in more than 350 paediatric cases, reports of adult patients with a vascular aetiology are extremely rare. In addition, the pathophysiological substrate of the syndrome remains unclear. We report an adult patient with PFS after surgical evacuation of a cerebellar bleeding. After 45 days of (akinetic) mutism, the patient’s cognitive and behavioural profile closely resembled the “cerebellar cognitive–affective syndrome”. A quantified SPECT study showed perfusional deficits in the anatomoclinically suspected supratentorial areas, subserving language dynamics, executive functioning, spatial cognition and affective regulation. We hypothesize that cerebello-cerebral diaschisis might be an important pathophysiological mechanism underlying akinetic mutism, cognitive deficits and behavioural–affective changes in adult patients with PFS.     

A child with mutism after bilateral thalamic infarction

The occurence of mutism after thalamic lesions has rarely been observed before adulthood. We report a 6.5-year-old girl who presented with sudden mutism with a decreased level of alertness. Her MRI revealed a T2-fluid attenuated inversion recovery hyperintensity in both thalami, which suggested bithalamic infarction in the territory of the thalamo-perforating arteries and, more specifically, the para-median territories. Mutism was followed by a deficit of speech initiation with slow and slurred speech. Her speech returned to normal at 3 months after the onset of symptoms. Thus mutism could be the equivalent of akinetic mutism limited to the speech sphere.     

Akinetic mutism: pharmacologic probe of the dopaminergic mesencephalofrontal activating system

Four children who exhibited akinetic mutism during the course of their neurologic diseases were treated with bromocriptine. Reversal of the akinetic mute states was evident in all patients. Pathways of the dopaminergic neurons are reviewed and a clinically useful mechanism which causes akinetic mutism is postulated.     

Akinetic mutism: a report of three cases

Objectives – To report 3 new cases of akinetic mutism, a clinical syndrome defined by silent immobility with preserved visual alertness not accountable by lesion of the areas and/or effector pathways of speech and voluntary movements. Material and methods – Anatomopathological studies were performed in Cases 1 and 2; clinical follow-up, EEG, angiography and CT scans in Case 3. Results – Case 1: Bipallidal necrosis; Case 2: Left pallidal necrosis with right frontoparietal cortico-subcortical infarction; Case 3: Striato-capsular infarction on the left side, involving the caudate nucleus and the anterior arm of the internal capsule, together with obstructive hydrocephalus. Conclusion – The roles of both globus pallidus and prefrontostriatal circuits in the onset of voluntary movements are discussed.     

A case of juvenile cerebral infarction caused by bilateral anterior cerebral artery dissection]

A 38-year-old man was admitted to our hospital with headache, dysarthria and paraparesis. Brain CT and diffusion MRI disclosed cerebral infarction at bilateral anterior cerebral artery (ACA) territories. His symptoms and signs deteriorated in several days despite intensive antithrombotic therapy, resulting in right hemiparesis, akinetic mutism, memory disturbance, change of personality, urinary incontinence, forced grasping, and starting delay of speech and motion. Cerebral angiography demonstrated occlusion with contrast pooling at the right ACA A2 portion. Stenosis and dilatation were found at left ACA A2 portion. An intimal flap was also demonstrated on serial angiography. This case was diagnosed as cerebral infarction caused by dissection of bilateral ACA. Although no definite primary arteriopathy was demonstrated, bilateral dissection could be occurred simultaneously.     

Spontaneous bilateral anterior cerebral artery occlusion resulting in akinetic mutism. A case report

An unusual case concerning a patient with akinetic mutism (AM) due to spontaneous bilateral anterior cerebral artery occlusion is reported. Brain CT scan revealed the presence of mild low density foci presenting an irregular enhancement, which followed the distribution of mesial frontal gyri and paracentral lobulus, bilaterally. Right and left carotid angiographies showed bilateral occlusion of the anterior cerebral artery. Our case is characterized by an exclusive localization of the infarction in the frontal cortex. This finding suggests that a limited damage involving the anterior cerebral arteries territory could be, on its own, responsible for the AM syndrome.     

Akinetic mutism from recurrent hydrocephalus: successful treatment with levodopa, bromocriptine and trihexyphenidyl

A case of akinetic mutism was reported with reference to a marked improvement by levodopa, bromocriptine and trihexyphenidyl. A 39-year-old male, first seen on February 2, 1981, had an occipitalgia, accompanied by nausea and vomiting. For several months before this consultation, the patient had suffered from asthenopia. Brain CT scan and cerebral angiogram demonstrated internal hydrocephalus due to aqueduct stenosis of unknown etiology. After a ventriculoperitoneal shunt operation on February 20, 1981, he completely recovered. Two years and a half after the shunt insertion he had no difficulty in his daily life. He reentered the hospital on December 21, 1983, because of personality change, mental deterioration and bradykinesia. Brain CT scan showed recurrent hydrocephalus resulting from shunt blockage. Following the shunt revision, hydrocephalus was resolved. Nevertheless, the patient did not return to his previous state. And he became bed-ridden, incontinent of urine, and unable to take fluids or foods, following which he went into a state of akinetic mutism. Other neurological findings were as follows: upward gaze palsy, impaired convergence, convergence nystagmus, plastic rigidity of neck and all four limbs, and diffuse hyperreflexia with right Babinski's sign. Abnormal involuntary movement was not seen. On March 27, 1984, levodopa therapy was instituted and on April 2, trihexyphenidyl was combined with levodopa. Shortly after administration of levodopa and trihexyphenidyl, akinetic mutism began to improve, but upward gaze palsy was not affected. He began to speak and could walk unassisted by the end of July.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

Differential diagnosis and classification of apathy

This paper discusses the definition of apathy, reviews its differential diagnosis, and proposes a classification for the conditions that may produce it. Apathy is defined as diminished motivation not attributable to diminished level of consciousness, cognitive impairment, or emotional distress. In its differential diagnosis, abulia, akinesia and akinetic mutism, depression, dementia, delirium, despair, and demoralization must be ruled out. Classification of apathy is organized in terms of its adaptive and functional consequences, its relationship to personality or to sociocultural or environmental events, and its association with psychiatric, neurological, and medical disorders. An approach to assessment and treatment is proposed.     

Akinetic mutism as a classification criterion for the diagnosis of Creutzfeldt-Jakob disease

OBJECTIVES—Among the classification criteria for the diagnosis of Creutzfeldt-Jakob disease, akinetic mutism is described as a symptom which helps to establish the diagnosis as possible or probable. Akinetic mutism has been anatomically divided into two forms—the mesencephalic form and the frontal form. The aim of this study was to delimit the symptom of akinetic mutism in patients with Creutzfeldt-Jakob disease from the complex of symptoms of an apallic syndrome and to assign it to the individual forms.
METHODS—Between April and December 1996, 25 akinetic and mute patients with Creutzfeldt-Jakob disease were consecutively examined. The patients were classified according to the definition of akinetic mutism by Cairns and secondly in accordance with the features constituting the complete picture of an appalic syndrome (defined by Gerstenbrand).
RESULTS—From 25 patients with definite Creutzfeldt-Jakob disease, 24 patients showed impoverishment of speech and, after a mean duration of four (range 1.1-11.2) months, almost complete absence of voluntary movements and speech. Seven patients were classified as being mute and akinetic and assigned to the mesencephalic form whereas 13 patients were classified as apallic. One patient was mute without being akinetic and four patients were comatose.
CONCLUSION—Diffuse brain damage underlies akinetic mutism in patients with Creutzfeldt-Jakob disease. The term can be used as a classification criterion for the diagnosis of Creutzfeldt-Jakob disease; however, it should be applied very carefully and delimited clearly from the apallic syndrome.

     

Akinetic mutism and progressive supranuclear palsy-like syndrome after the shunt of an obstructive hydrocephalus. Successful treatment with bromocriptine: 2 cases

Two cases of obstructive hydrocephalus who suffered multiple shunt failures and shunt revisions are presented. The patients developed after the neurochirurgical treatment a clinical syndrome of akinetic mutism followed by a Progressive Supranuclear Palsy-like (PSP) syndrome. The akinetic mutism and the PSP-like syndrome were remarkably improved with bromocriptine.     

Dementia and disturbance of consciousness in thalamic degeneration

A 53-year-old male with thalamic degeneration is presented. He had double vision, cerebellar signs, and pyramidal and extrapyramidal tracts signs in addition to hypersomnia, decrease in spontaneity and attention, and impairment of memory as psychic symptoms. These signs and symptoms were progressive, and he subsequently developed akinetic mutism and died of pneumonia 17 months after the onset of the disease. His clinical diagnosis was considered as Gerstmann-Str?ussler syndrome due to progressive dementia, cerebellar signs and the other signs mentioned above. The postmortem pathological investigations, however, revealed thalamic degeneration. The pathological observations showed marked loss of nerve cells and glial proliferation in the medial and anterior nuclei of thalamus. The same pathological changes were more or less demonstrated in the pulvinar, the periaqueductal gray matter of midbrain, inferior olivary nucleus, the medial parts of globus pallidus, the substantia nigra and the dentate nucleus. In the early stage of the clinical course, it was difficult to know whether the main symptoms were caused by dementia or by the disturbance of consciousness. Retrospective considerations, however, showed that dementia had appeared at first, and subsequently the disturbance of consciousness had joined. As the result, it seems that they finally caused akinetic mutism. It is known as thalamic dementia that in the cerebrovascular disease the lesions in the medial and anterior parts of bilateral non-specific thalamic nuclei cause dementia.(ABSTRACT TRUNCATED AT 250 WORDS)     

Bilateral, symmetrical and simultaneous putamino-capsulo-caudal infarction

The first case, to our knowledge, of bilateral, symmetric and simultaneous infarction in the basal territory of the middle cerebral arteries is reported. The patient presented with a pseudo-rostral brainstem syndrome, including loss of oculo-vestibular reflexes, severe tetraparesia and akinetic mutism-like disturbance of consciousness. The lesions were due to embolism, caused by atrial fibrillation, occluding the ostium of the lenticulostriate arteries. It involved only the putamino-capsulo-caudal regions, concerning the whole anterior and posterior dorsal capsules. The term tetraparetic mutism is proposed to describe this new clinico-pathological syndrome, which is the sum of a capsular tetraparesia, due to the lesion of the geniculate and cortico-spinal fibres at the level of the genu and posterior limbs, and of a capsular akinetic mutism, due to the lesion of the reticular thalamo-cortical tracts at the level of the anterior limbs. These total capsular infarcts moreover involved all the oculomotor cortico-reticular tracts, which could explain the absence of oculo-vestibular reflexes.