[Akinetic mutism (author's transl)].
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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.
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Two cases of spontaneous cerebral ventriculostium are presented. The first case is that of a 3 year-old girl with a thumb-sized soft scalp tumor of the occipital region (dural hypertrophy) and hydroencephalodysplasia (Picaza). PVG revealed noncommunicating hydrocephalus with asymmetrical deformity of the lateral ventricle and agenesis of corpus callosum (Fig. 1). Ventriculoatrial shunt was performed. Three years passed under the useful life when she readmitted to our clinic complaining headache, nausea and vomiting. On the first hospital day she fell into respiratory arrest accompanied with coma after the tonic convulsion, and eventually, she died on the fourth hospital day. Postmortem examination revealed spontaneous cerebral ventriculostium which communicated with the posteromedial trigone of the left lateral ventricle (Fig. 3). Combined other malformations such as dysgenesis of the corpus callosum and only one anterior cerebral artery, etc. were found. The second case is that of a young adult, a 22 year-old male with rapidly progressing intracranial hypertension. PVG revealed marked dilatation of the lateral and the third ventricle, non-filling of the aqueduct and spontaneous cerebral ventriculostium which communicated with the posterior part of the third ventricle (Fig. 4). And insidiously he fell into akinetic mutism. After suboccipital exploratory craniotomy and ventriculo-peritoneal shunt akinetic mutism improved gradually, and he was discharged on foot after 7 months. PEG performed on June 8, 1973, showed no evidence of aqueduct obstruction and injected air passed from the fourth ventricle to the third one smoothly. He lives on now under a useful condition. These 2 cases are the first report on literatures in Japan, but presumably there must be many other cases. Since W. H. Sweet reported his own two cases of spontaneous cerebral ventriculostium on 1940, more than thirty cases have been published on literatures. However, there are found various expressions to describe the same condition (Table 1). We would like to propose that the most suitable expression is "ventriculostium" not only in deference to the originality of W. H. Sweet but also not to confuse this pathogenetic state with other similar conditions. The author's next interest is the chronological fact that from W. H. Sweet (1940) to A. Torkildsen (1948), all but one ostiums reported situated at the posteromedial trigone of the lateral ventricle, whereas after A. Torkildsen, they were found at the posterior part of the third ventricle in many cases. The reason is unknown. It would appear that three main conditions are necessary for the development of ventricluostium just beneath the tentorium. The first, there must be increased pressure within the lateral or the third ventricle. The second essential feature is the lack of any large space occupying lesion in the the infratentorial space. The third, there must be wider space between the tentorial incisura and the brain stem.
The clinical and pathological data of a 48 year old patient who survived 40 days are reported. Her disturbance of consciousness corresponded to an apallic syndrome, which 12 days later bacame akinetic mutism. Symmetrical softening involving the medial thalamic nuclei from the plane of the corpora mamillaria to the red nuclei was found. The ischemic lesion might be explained by transient circulatory collapse combined with hypoplasia of the vertebrobasialr arteries. On the EEG slight irregular alpha activity was recorded (alpha coma) and external stimuli elicited theta-delta waves (paradox activation). A survey of the literature of akinetic mutism is included and the correlation between non-hypnoid unconsciousness and EEG is discussed.
Patients with herpesvirus infections were given intravenous injections of 10-20 mg of adenine arabinoside (ara-A)/kg per day. When given the higher dosage, some patients with chronic hematologic conditions showed mild to moderate additional depressions in the level of hemoglobin. The number of neutrophils and platelets did not decrease, even when numbers were low at the onset of treatment with ara-A. Two patients with Hodgkin's disease who received 20 mg of ara-A/kg per day developed a transient motor aphasia resembling akinetic mutism. With the regimens of ara-A used and challenge inocula of approximately 50 plaque-forming units of virus, the minimal inhibitory concentrations of ara-A and hypoxanthine arabinoside for herpesviruses are usually not achieved in serum but may be attained in body fluids (urine and vesicular fluid). Antiviral activity in vesicular fluids is likely to involve a combination of ara-A, hypoxanthine arabinoside, and interferon.
An autopsy case of multiple system degeneration is characterized by the following; (1) progressive dementia and abnormal sleep patterns, followed by Parkinsonian symptoms with terminal akinetic mutism; (2) severe symmetrical degeneration in the thalamus, particularly nucl. medialis thalami, the reticular formation of the brain stem, also the pallidonigral, pallido-Luysian and dentato-rubral systems. As far as we known, there is no case in the literature, of combined system degeneration, which shows such a wide anatomical range of lesions as the present one. Clinico-pathological correlation between dementia and degeneration of the thalamus, and between abnormal sleep-consciousness mechanism and degeneration of the reticular formation are discussed. The extrapyramidal symptoms are discussed from the clinico-pathological aspect. In addition, peculiar eosinophilic bodies are described, which were most frequently found in the putamen.
Nine of 48 adult patients who underwent orthotopic liver transplantation developed significant clinical neurological abnormalities recognized shortly after operation. Decrease in consciousness occurred with resultant coma, focal and generalized seizures and the occasional appearance of a state of akinetic mutism. Neuropathological abnormalities consisted of multifocal areas of infarction in cerebral cortex and basal ganglia in five patients, central pontine myelinolysis in five (often more extensive than usually reported), Wernicke's encephalopathy in three, glial nodules in two, and fungal abscesses in one. Alzheimer II astrocytosis was found in all brains available for retrospective study. There was direct evidence in two of the patients that air embolization from the homografts had occurred. Correlation of this with the brain infarcts in these and other cases seems reasonable. The ease with which air passed to the systemic circulation is explicable by the right to left venous--arterial shunts that are common in chronic liver disease. With the delination of this cause for the neurologic complications, measures to prevent it in future cases have been described.