Akinetic Mutism and Bilateral Anterior Cerebral Artery Occlusion
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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.34.6.693 on 1 December 1971. Downloaded from J. Neurol. Neurosurg. Psychiat., 1971, 34, 693-698 Akinetic mutism and bilateral anterior cerebral artery occlusion FRANK R. FREEMON From the Department of Neurology, Marquette School of Medicine, Milwaukee, Wisconsin, U.S.A. SUMMARY Three cases of bilateral anterior cerebral artery occlusion are presented with akinetic mutism. The anatomical distribution of the infarction in these patients combined with cases in the literature suggests that this syndrome can have a localizing value for the clinician. If increased intraventricular pressure is not present, the clinician can suspect a bilateral lesion of cingulate gyrus, medial nuclei of basal ganglia, and/or anterior and reticular nuclei of the thalamus. ment elicitable was a withdrawal Akinetic mutism is a disorder of consciousness slight of each limb to guest. Protected by copyright. characterized by unresponsiveness but with the deep pain stimulation. However, if the patient's arm was superficial appearance of alertness. The patient's held over his face and allowed to drop, it usually fell in eyes are open and he may seem to look at the such a way as to miss his face. Tendon reflexes were bi- laterally symmetrical and hyperactive, particularly in the examiner but he neither speaks nor moves, nor is the lower extremities with sustained clonus at both ankles. examiner able to communicate with the patient. Bilateral Babinski signs were present, as were snout and Poppen (1939) and Dandy (1946) each reported a grasp reflexes. The patient would look at the examiner case of akinetic mutism after operative sacrifice of and would follow him with his eyes. Several attempts to both anterior cerebral arteries. Skultety (1968) communicate with the patient using eye movements or described a patient who developed akinetic mutism eye blinks as signals were entirely unsuccessful. Within after the clipping of the right anterior cerebral artery an hour after the insult, an electroencephalogram was during the removal of a falx meningioma with performed which showed low voltage 2-2i Hz generalized apparent postoperative thrombosis in the left activity. A lumbar puncture showed an opening pressure anterior of 140 mm CSF and a closing pressure of 120 mm CSF; cerebral artery. This communication de- glucose was 77 mg and protein 45 mg/100 ml. There were scribes three cases of akinetic mutism associated no cells in the CSF and there was no xanthochromia. with spontaneous bilateral anterior cerebral artery The next morning the patient had lost his superficial occlusion and discusses the neuroanatomical sub- appearance of alertness. His eyes were closed and his strate which may underlie the appearance of this musculature was flaccid unless he was stimulated at which syndrome. time he became rigidly opisthotonic with extension of legs and arms, with flexion at the wrists. He had Cheyne- CASE 1 Stokes respiration with an apnoeic period of 20 seconds. http://jnnp.bmj.com/ At this time the pupils remained equal and reactive, A 68 year old retired depressed white male was admitted extraocular movements were intact as estimated by the to the surgical service because of a self-inflicted minor doll's head manoeuvre, and caloric examination showed gunshot wound to the chest. The only significant past conjugate tonic deviation of the eyes toward the side of history was a myocardial infarction 13 years previously. the cold water. The patient died 10 days later. After successful treatment of his chest wound the patient was transferred to the psychiatric service because of con- NEUROPATHOLOGICAL FINDINGS External examination tinued suicidal thoughts and statements. One morning a revealed bilateral softening from the frontal pole to the week later he awoke feeling unusually 'groggy' as he told inferior occipital region with slight clouding and thicken- on September 29, 2021 by his room-mate. On his way to the bathroom the patient ing of the overlying meninges. Coronal sections showed fell and could not arise. When he was examined his eyes severe infarction on the left, involving the entire fronto- were open but he could not speak or follow commands. parietal and temporal cortex extending through sub- The doll's head manoeuvre showed extraocular move- cortical white matter to the basal ganglia, including the ments to be full. Pupils were equal and reactive to light. head of the caudate nucleus but sparing the thalamus. Gag reflex was present as were corneal reflexes. A slight The area of infarction was more patchy on the right but plastic rigidity was present in all limbs. The only move- extended throughout the area of distribution of the .r 6J9K.1 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.34.6.693 on 1 December 1971. Downloaded from 694 Frank R. Freemon anterior and middle cerebral arteries. The occipital regions, the cerebellum, and the brain-stem were normal both grossly and microscopically. Microscopic examina- tion in the infarcted area showed loss of parenchyma, necrosis of vessels, and accumulation of polymorpho- .4 , ..;... nuclear leucocytes. The blood vessels of the circle of Willis were markedly atherosclerotic. The right internal carotid artery was small in size and was occluded by an ..i old partially organized thrombus which showed extensive AX~'iI recanalization on microscopic examination. The left carotid artery near its bifurcation was occluded by a fresh thrombus which extended upward into the middle and anterior cerebral arteries as well as the anterior com- municating and right anterior cerebral arteries. Micro- scopic examination revealed this to be a mixture of old and recent thrombosis with partial recanalization. CASE 2 A 72 year old retired white carpenter in good health except for occasional dizzy spells suddenly became un- conscious after supper, falling on the couch. The un- responsive patient was kept at a local nursing home for the next seven weeks. An electroencephalogram (EEG) during this period was locally interpreted as normal but guest. Protected by copyright. was not available for review. When transferred to a Veteran's Administration hospital, the patient had an alert appearance but was unresponsive. His eyes were usually open and moved spontaneously as though he were looking around the room; they did not follow a moving object or person. He would not respond to auditory commands including those associated with eye move- ments. No attempt was made to communicate with the FIG. 1. The distribution of infarcted area in case 2. patient by means of eye blinks or movements. To super- ficial tactile stimulation the patient gave no response but to deep pain stimulation on the right side of the body or face only, he made writhing and purposeless movements CASE 3 of the right arn and leg. Unrelated to stimulation or commands, the patient made occasional, apparently A 62 year old Negro diabetic pulpwood labourer on spontaneous, slight movements of the right arm. Muscle insulin for 10 years was hospitalized in diabetic keto- tone was markedly increased in the right arm and leg. acidosis. Seven years before admission the patient awoke There was a left Hoffman sign, a left Babinski sign, and with a slight left hemiparesis without any other symptoms; hyperactive tendon reflexes on the left. A snout reflex was this left-sided weakness had improved but never com- present as was a grasp reflex ofthe right hand. The patient pletely cleared. For the three days before admission, the died the following day. patient increased his food and water intake without a change in his daily dose of 70 units of lente-insulin and in http://jnnp.bmj.com/ NEUROPATHOLOGICAL FINDINGS External examination the absence of infection or trauma. On the day of admis- showed a sunken haemorrhagic area in the distribution of sion, he became increasingly confused and finally became the right mniddle cerebral artery. Coronal sectioning re- unconscious. No hypoglycaemia was noted during insulin vealed an infarction in the right anterior and middle and fluid treatment of the ketoacidosis. On the third cerebral artery distribution involving frontoparieto- hospital day, the patient opened his eyes but remained temporal cortex, basal ganglia, including the head of the unresponsive. His eyes moved conjugately as though he caudate nucleus, and internal capsule but sparing were looking about the room but without following thalamus. On the left a cystic degeneration involved the moving objects. The patient failed to respond to any head of the caudate nucleus, the medial putamen, and auditory or visual command, except on one occasion on September 29, 2021 by the anterior limb of the internal capsule sparing overlying when he opened his mouth after the oral command to cortex. There were no other abnormalities. Microscopic perform this movement. Pain stimulation caused slight examination of the infarcted areas showed tissue necrosis withdrawal movements of the appropriate extremity. with reactive gliosis. Examination of cerebral vessels re- Pupils were equal and reactive. Corneal reflexes were bi- vealed moderateatherosclerosisbut no apparent occlusion laterally absent. The 'doll's head' manoeuvre showed or thrombosis. Figure 1 shows the pathological specimen complete conjugate eye movements. Caloric stimulation in this patient. caused conjugate tonic deviation of the eyes toward the J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.34.6.693 on 1 December 1971. Downloaded from Akinetic mutism and bilateral anterior cerebral artery occlusion 695 side of the cold water. Muscle tone was normal. Tendon reflexes were decreased and abdominal reflexes were absent. A Babinski sign was present on the left but not on the right. A lumbar puncture showed normal pressure with CSF protein of 54 and glucose of 109 mg/100 ml. An EEG showed diffuse theta and delta activity. The patient died five weeks after admission without ever regaining consciousness. About three weeks before he died, he closed his eyes and lost the alert appearance he had demonstrated previously.