Episodically Remitting Akinetic Mutism Following Subarachnoid Hemorrhage
Publication: The Journal of Neuropsychiatry and Clinical Neurosciences
Abstract
Akinetic mutism is characterized by alertness with near complete absence of volitional activity. The authors report a case of episodically remitting akinetic mutism following subarachnoid hemorrhage.
Prolonged unresponsive states due to a variety of neuropsychiatric conditions have been reported frequently in the literature.1–4 These conditions are characterized by some combination of excitement, motoric immobility, extreme negativism, mutism, mannerisms, stereotypies, echolalia, and echopraxia. The typical course is one of rapid recovery, although often there are residual psychiatric or medical symptoms. Although there are many cases of catatonia and akinetic mutism following subarachnoid hemorrhage,1–3,5,6 we could find no report of an akinetic mute state that remitted episodically only to return to full mute immobility after 1 or 2 days.
CASE REPORTB., a 52-year-old former electrician, was admitted to the psychiatry service with an absence of spontaneous activity. He had been well, with no psychiatric or medical history, until at age 48 he suffered a ruptured basilar artery aneurysm, which was surgically treated. The patient was comatose for approximately 4 weeks, then gradually regained consciousness. Rehabilitation brought return of his strength, stamina, and ability to ambulate. He was left, however, without volitional activity.Seventeen months later, the patient's wife reports that he experienced his first “awakening.” B.'s mental status erupted into its premorbid state, with full return of his demeanor and affect just as they were before the stroke. He was inquisitive about changes in his life, specifically the sale of his beloved fishing boat, but would not believe he had suffered a stroke. After sleeping through the night, the patient was once again akinetic and mute.After another year, the patient had his first grand mal seizure. Within 3 days, his persona emerged again. Four more times over the next year his affect returned on the heels of a seizure. The potential connection led the patient's wife to seek psychiatric evaluation for possible ECT and to stop his phenytoin.Upon admission, the patient would respond to questions and follow simple commands, but he initiated no spontaneous action. He stayed where placed, with no effort to move, eat, or toilet himself. He would stare blankly and emotionlessly at nothing, with his hands always placed on his thighs. Neurologic examination revealed a deficit in downward gaze bilaterally and hyperactive patellar reflexes. He walked with a parkinsonian gait and minimal arm swing, but there was no tremor. There was no other abnormality of strength or sensation, and he exhibited no pathological reflexes. Cognitively, the patient was able to score 20 out of 30 on the Mini-Mental State Examination, losing points on orientation and recall.Formal neuropsychological testing included Wechsler Adult Intelligence Scale–Revised, Object Memory Selective Reminding Test, Boston Naming Test, Controlled Oral Word Association Test, Visual Organization Test, and Apraxia Screening Exam. These revealed extremely impaired cognitive function. Verbal IQ was in the low normal range, fund of general information was below average, and verbal abstraction was poor. Object-naming memory was not preserved, and generative naming was extremely impaired. The patient showed severe impairment for recent memory and reduced speed of information processing.CT scan showed postoperative changes along with encephalomalacic changes in the right temporal pole and chronic ischemic focal changes in the basal ganglia bilaterally and in the left thalamus. There was no hydrocephalus. EEG was abnormal, with slow occipital rhythm and background activity suggesting a mild, diffuse disturbance in cerebral function. There was also a focal disturbance in the right temporal region consisting of intermittent, low-voltage slow activity, but no epileptiform activity.A cerebral angiogram revealed a residual, 3–4-mm aneurysm at the basilar tip. It was therefore considered unsafe to proceed with ECT for fear of inducing another hemorrhage. Medical management was attempted with trials of bromocriptine, methylphenidate, and parenteral lorazepam. These brought no clinical improvement and had significant side effects.After careful consideration, the patient's wife elected to proceed with intravascular embolization of the residual aneurysm to allow a trial of ECT. Of note was another arousal, which occurred postoperatively. This episode was preserved on videotape.Almost 3 years after the initial stroke, B. was admitted to this institution for a trial course of ECT. Six convulsions were induced over a 2-week period. After the first two, the patient awakened much as before. Unfortunately, he returned to his akinetic mute state for the remainder of the hospitalization despite the ongoing ECT. At discharge, there was little change from his first admission 7 months earlier.
DISCUSSION
As in the case report by Scott et al.,7 this patient has proven difficult to classify. The similarities between catatonia and akinetic mutism challenged us to explore the diagnostic criteria more closely. In the recent official position paper of the American Congress of Rehabilitation Medicine,8 akinetic mutism was defined by the presence of eye opening and spontaneous visual tracking, little or no spontaneous speech or movement, infrequent following of commands, and absence of a causative neuromuscular disturbance. These criteria more closely match the patient than those of catatonia, with its associated rigidity, posturing, waxy flexibility, and bouts of excitement. The patient would, however, meet the very broad DSM-IV criteria for Catatonic Disorder Due to a General Medical Condition.
A limited list of organic causes of akinetic mutism includes vascular accidents, mass lesions, multiple sclerosis, trauma, and encephalitis lethargica.2–7 We could not find a case, however, with vacillation between akinetic mutism and premorbid personality.
The task for the authors is to generate a plausible explanation for these episodes. The patient initially presented to R.C. for ECT because of the postictal timing of his recovered alertness and affect. Indeed, it is inviting to explain this syndrome as epileptiform because of the suddenness of the transformations and their association to his tonic-clonic seizures. The EEG done during the patient's baseline state did show some focal, though nonepileptiform, activity.
Presence of this focal activity begets the question of nonconvulsive status epilepticus (NCSE) causing these lengthy periods of akinesis. This is a compelling hypothesis because NCSE, or psychomotor status, has been reported to cause similar symptoms,9–11 as well as almost any other psychiatric symptom complex.12 There have even been cases of NCSE with seizure duration of 7.5 months,13 and EEG may fail to show characteristic epileptiform changes.9,11 There is also strong evidence against this diagnosis, however. The aforementioned EEG devoid of epileptiform activity argues against NCSE. Additionally, the patient's failure to respond to phenytoin, parenteral lorazepam, or ECT, all potent anticonvulsants, makes this diagnosis unlikely, though a refractory case remains possible.
An intermittently reversing ischemic etiology was considered, but this was considered impossible because of the rapid loss of viability of cortical tissue during periods of hypoxia. Functional psychiatric disorder was felt unlikely with this patient's lack of premorbid psychiatric history and the absence of other evidence for primary psychiatric illness. Infection, mass lesion, and metabolic derangement were all eliminated from the differential by examination, laboratory evaluation, and imaging analysis.
The reader will no doubt notice that we have not yet provided “The Answer” to this puzzle. We would like to offer one more potential cause for this patient's condition. There could exist an as yet undiscovered neurochemical gating system, analogous to the reticular activating system, which when lesioned prevents operation of the parts of the cortex necessary for purposeful, intentional activity. In this patient, an undetermined stimulus may be able to overcome the threshold of this damaged circuit, allowing fleeting activation of B.'s cortex and thereby his personality. Detailed imaging of the brainstem, using MRI scanning, would have been extremely valuable to further elucidate this hypothesis, but this was precluded by the aneurysm clip in place.
Despite its uncertainty, this case remains instructive in terms of the thought process involved in pursuing a differential diagnosis and the challenges encountered while treating neuropsychiatric patients.
ACKNOWLEDGMENTS
This work was previously presented at the annual meeting of the British Neuropsychiatric Association, Cambridge, England, July 1997.
References
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Published online: 1 February 1999
Published in print: February 1999
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