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Prolonged abulia following putaminal hemorrhage
Prolonged Abulia Following Putaminal Hemorrhage Nages Nagaratnam,
MD,
Sue Fanella, BSc (Speech Path), Sumana Gopinath and Annabel Goodwin, MB
MB,
Abulia, akinetic mutism, and other conditions causing reduced activity and slowness are a continuum of severity of behavior. Unilateral lesions usually cause transient symptoms. This article describes a patient with prolonged abulia lasting 12 weeks after aspontaneous left putaminal hemorrhage. He developed seizures that could be a contributing factor. The pathophysiologic mechanisms are discussed. Key Words: Abulia—Akinetic mutism—Putaminal hemorrhage. Copyright © 2001 by National Stroke Association
Terms such as apathy, abulia, and akinetic mutism (AM) are used to designate behavioral abnormalities relating to reduced activity and slowness. Several investigators proposed that these clinical disorders are simply a continuum of severity of reduced behavior, and AM may be the extreme form. The term abulia was used by Auerbach1 in 1902, and later was called akinesia. Fisher2 preferred the term abulia to AM for several reasons, and he used the term abulia to describe the full spectrum of abnormalities. Abulia was characterized by a reduction in speech, spontaneous activity, prolonged latency in responding to questions, and a lack of persistence with tasks.2 Neuroanatomic analysis in patients with reduced behavioral abnormalities (e.g., abulia and AM) suggest that, anatomically, there does not seem to be any distinct impairments in these 2 disorders. Unilateral lesions with AM usually cause transient symptoms.3 We describe a patient with prolonged duration of abulic manifestations after a putaminal hemorrhage. We discuss the likely underlying pathophysiologic mecha-
From the Department of Medicine (Aged Care and Rehabilitation Services), Blacktown-Mount Druitt Health, Blacktown, New South Wales 2148 Australia. Received April 10, 2000; accepted March 9, 2001. Address reprint requests to Nages Nagaratnam, MD, Department of Medicine (Aged Care and Rehabilitation Services), Blacktown Hospital, Blacktown, New South Wales 2148, Australia. Copyright © 2001 by National Stroke Association 1052-3057/01/1002-0002$35.00/0 doi:10.1053/jscd.2001.24662
92
nisms and whether the diverse locations seen in patients with abulia and AM have a common neural substrate.
Case Report A 79-year-old man was seen on September 30, 1999, with slurred speech, right facial asymmetry, and subtle weakness of the right side. On the following day, he was alert, cooperative and able to follow simple commands, but he had difficulty with prolonged delay in providing responses to questions. His volitional swallowing was strong but his chewing (soft fruit and biscuit) required effort. He ate and drank very slowly and kept remnants of food in his mouth. In spite of minimal weakness of his right lower limb, he could walk only with the assistance of 2 people, and he had urinary incontinence. He remained inactive in both conversation and activity. Seven days after presentation, he was assessed using the Boston Diagnostic Aphasia Examination (BDAE). On auditory comprehension, he was able to follow simple instructions but performance and response was slow with longer instructions and with yes or no questions. He was 80% correct on general knowledge. Expression was 75% correct for naming objects with 50% perseversation on previous response. Constant prompting was needed. The patient’s automatic speech was good when he was asked to name days of the week, but he performed only 50% of the counting task. He was able to read instructions accurately but had difficulty following longer written instructions. On the morning of day 16, the patient was very drowsy and confused over several hours. He had similar episodes a week and 10 days later and was given
Journal of Stroke and Cerebrovascular Diseases, Vol. 10, No. 2 (March-April), 2001: pp 92-93
ABULIA PUTAMINAL HEMORRHAGE
Figure 1. (A) A computed tomography scan on day 1 shows an extensive hemorrhage in the left putamen and extending towards the internal capsule. (B) A computed tomography scan on day 18 shows a hypodense area involving the left putamen.
treatment of dilantin sodium after which there were no recurrence of the events. Neurologically, at the end of 2 weeks, the patient remained slow to respond. He lacked spontaneity with impassive facies and a depressed affect. Muscle strength and bulk were normal. There was no rigidity. Meaningful bimanual tasks, such as clapping the hands, were possible but slow. Alternate, repetitive, and 5-finger movements were possible with poor sequencing on both sides. One finger tapping was normal and equal in both hands. The patient still required assistance of 1 person to walk. Four weeks after onset, he remained noncommunicative and poorly responsive. A week later, he could walk 10 meters in 26 seconds with standby assistance and requiring encouragement and persuasion. On December 21, 1999, the patient could walk with a pick-up frame and was more responsive to conversation and to activities of basic self care. The computed tomography scan on day 1 showed an extensive hemorrhage in the left putamen, which extended towards the internal capsule. A repeat scan on day 18 showed a hypodense area in the left putamen (Fig 1).
Discussion We have described a patient with prolonged reduced behavior after a spontaneous left putaminal hemorrhage who satisfied the criteria for abulia.2 The patient was abulic for more than 3 months. According to Caplan et al.,4 the anatomy of abulia has not been systematically studied. Abulia occurs after lesions either in the frontal lobes and underlying structures or in the thalamus or upper brain stem.2 Caplan et al.4 described 10 abulic patients with predominant involvement of the left caudate nucleus of whom 4 involved the anterior body, 4 involved only the
93
caudate, and only 1 involved the putamen. Fisher’s abulic patients had lesions in the frontal lobes and underlying structures, the upper brain stem, and the thalamus. Experiments on animals and humans have given some input regarding the pathogenesis. Bilateral electrical lesions in the caudate and anterior portion of the putamen of adult monkeys have resulted in slowness, poor attention, and proneness to stand and stare for as long as 7 weeks.5 Unilateral lesions usually give rise to symptoms that are short lived. One explanation for the prolongation of the symptoms could be possible hypoxic injury after the seizures, and another explanation is the large volume of the hemorrhage. Depending on the clinical picture, AM is subdivided into 2 types based on the anatomic location of the lesion.6 One type is known as hyperpathic AM and is associated with bilateral frontal damage. The other type is related to the mesencephalic region and is described as apathetic AM or somnolent mutism.6 Behavioral changes are linked with the frontal-subcortical circuitry7 and are mediated by transmitters and modulators. Five circuits have been described8 and they originate in the frontal lobes with projections to the striatal structures (caudate, putamen, and ventral striatum) with connections from the striatum to the globus pallidus, substantia nigra, and projections from them to the specific thalamic nuclei, and, finally, they connect with the frontal lobe.7,8 They share the common structures, namely the frontal lobe, striatum, globus pallidus, substantia nigra, and the thalamus. Dysfunction of the fronto-subcortical circuitry could cause a wide range of behavioral changes, and the cingulate syndrome is characterized by AM, aspontaneity, and marked apathy.7
References 1. Auerbach S. Beitrag zur diagnostik der Geschwulste des Stirnhirn. Dtsch Z Nervenheilkd 1902;22:312-332. 2. Fisher CM. Abulia minor vs agitated behaviour. Clin Neurosurg 1983;3:319-331. 3. Damasio H, Damasio AR. Lesion analysis in neuropsychology. New York, NY: Oxford-University Press, 1989: 60-61. 4. Caplan LR, Schmchmann D, Kasi CS, et al. Caudate infarcts. Arch Neurol 1990;47:133-142. 5. Denny-Brown D. The basal ganglia and the relation to disorders of movement. New York, NY: Oxford University Press, 1962. 6. Segarra JM. Cerebral vascular disease and behaviour. I. Syndrome of the mescencephalic artery (basilar artery bifurcation). Arch Neurol 1970;22:408-418. 7. Cummnigs JL. Fronto-subcortical circuits and human behaviour. Arch Neurol 1993;50:873-880. 8. Alexander GE, Delaney MR, Strick PL. Parallel organisation of functionally segregated circuits linking basal ganglia and cortex. Ann Rev Neurosci 1990;9:357-381.
MD,
Sue Fanella, BSc (Speech Path), Sumana Gopinath and Annabel Goodwin, MB
MB,
Abulia, akinetic mutism, and other conditions causing reduced activity and slowness are a continuum of severity of behavior. Unilateral lesions usually cause transient symptoms. This article describes a patient with prolonged abulia lasting 12 weeks after aspontaneous left putaminal hemorrhage. He developed seizures that could be a contributing factor. The pathophysiologic mechanisms are discussed. Key Words: Abulia—Akinetic mutism—Putaminal hemorrhage. Copyright © 2001 by National Stroke Association
Terms such as apathy, abulia, and akinetic mutism (AM) are used to designate behavioral abnormalities relating to reduced activity and slowness. Several investigators proposed that these clinical disorders are simply a continuum of severity of reduced behavior, and AM may be the extreme form. The term abulia was used by Auerbach1 in 1902, and later was called akinesia. Fisher2 preferred the term abulia to AM for several reasons, and he used the term abulia to describe the full spectrum of abnormalities. Abulia was characterized by a reduction in speech, spontaneous activity, prolonged latency in responding to questions, and a lack of persistence with tasks.2 Neuroanatomic analysis in patients with reduced behavioral abnormalities (e.g., abulia and AM) suggest that, anatomically, there does not seem to be any distinct impairments in these 2 disorders. Unilateral lesions with AM usually cause transient symptoms.3 We describe a patient with prolonged duration of abulic manifestations after a putaminal hemorrhage. We discuss the likely underlying pathophysiologic mecha-
From the Department of Medicine (Aged Care and Rehabilitation Services), Blacktown-Mount Druitt Health, Blacktown, New South Wales 2148 Australia. Received April 10, 2000; accepted March 9, 2001. Address reprint requests to Nages Nagaratnam, MD, Department of Medicine (Aged Care and Rehabilitation Services), Blacktown Hospital, Blacktown, New South Wales 2148, Australia. Copyright © 2001 by National Stroke Association 1052-3057/01/1002-0002$35.00/0 doi:10.1053/jscd.2001.24662
92
nisms and whether the diverse locations seen in patients with abulia and AM have a common neural substrate.
Case Report A 79-year-old man was seen on September 30, 1999, with slurred speech, right facial asymmetry, and subtle weakness of the right side. On the following day, he was alert, cooperative and able to follow simple commands, but he had difficulty with prolonged delay in providing responses to questions. His volitional swallowing was strong but his chewing (soft fruit and biscuit) required effort. He ate and drank very slowly and kept remnants of food in his mouth. In spite of minimal weakness of his right lower limb, he could walk only with the assistance of 2 people, and he had urinary incontinence. He remained inactive in both conversation and activity. Seven days after presentation, he was assessed using the Boston Diagnostic Aphasia Examination (BDAE). On auditory comprehension, he was able to follow simple instructions but performance and response was slow with longer instructions and with yes or no questions. He was 80% correct on general knowledge. Expression was 75% correct for naming objects with 50% perseversation on previous response. Constant prompting was needed. The patient’s automatic speech was good when he was asked to name days of the week, but he performed only 50% of the counting task. He was able to read instructions accurately but had difficulty following longer written instructions. On the morning of day 16, the patient was very drowsy and confused over several hours. He had similar episodes a week and 10 days later and was given
Journal of Stroke and Cerebrovascular Diseases, Vol. 10, No. 2 (March-April), 2001: pp 92-93
ABULIA PUTAMINAL HEMORRHAGE
Figure 1. (A) A computed tomography scan on day 1 shows an extensive hemorrhage in the left putamen and extending towards the internal capsule. (B) A computed tomography scan on day 18 shows a hypodense area involving the left putamen.
treatment of dilantin sodium after which there were no recurrence of the events. Neurologically, at the end of 2 weeks, the patient remained slow to respond. He lacked spontaneity with impassive facies and a depressed affect. Muscle strength and bulk were normal. There was no rigidity. Meaningful bimanual tasks, such as clapping the hands, were possible but slow. Alternate, repetitive, and 5-finger movements were possible with poor sequencing on both sides. One finger tapping was normal and equal in both hands. The patient still required assistance of 1 person to walk. Four weeks after onset, he remained noncommunicative and poorly responsive. A week later, he could walk 10 meters in 26 seconds with standby assistance and requiring encouragement and persuasion. On December 21, 1999, the patient could walk with a pick-up frame and was more responsive to conversation and to activities of basic self care. The computed tomography scan on day 1 showed an extensive hemorrhage in the left putamen, which extended towards the internal capsule. A repeat scan on day 18 showed a hypodense area in the left putamen (Fig 1).
Discussion We have described a patient with prolonged reduced behavior after a spontaneous left putaminal hemorrhage who satisfied the criteria for abulia.2 The patient was abulic for more than 3 months. According to Caplan et al.,4 the anatomy of abulia has not been systematically studied. Abulia occurs after lesions either in the frontal lobes and underlying structures or in the thalamus or upper brain stem.2 Caplan et al.4 described 10 abulic patients with predominant involvement of the left caudate nucleus of whom 4 involved the anterior body, 4 involved only the
93
caudate, and only 1 involved the putamen. Fisher’s abulic patients had lesions in the frontal lobes and underlying structures, the upper brain stem, and the thalamus. Experiments on animals and humans have given some input regarding the pathogenesis. Bilateral electrical lesions in the caudate and anterior portion of the putamen of adult monkeys have resulted in slowness, poor attention, and proneness to stand and stare for as long as 7 weeks.5 Unilateral lesions usually give rise to symptoms that are short lived. One explanation for the prolongation of the symptoms could be possible hypoxic injury after the seizures, and another explanation is the large volume of the hemorrhage. Depending on the clinical picture, AM is subdivided into 2 types based on the anatomic location of the lesion.6 One type is known as hyperpathic AM and is associated with bilateral frontal damage. The other type is related to the mesencephalic region and is described as apathetic AM or somnolent mutism.6 Behavioral changes are linked with the frontal-subcortical circuitry7 and are mediated by transmitters and modulators. Five circuits have been described8 and they originate in the frontal lobes with projections to the striatal structures (caudate, putamen, and ventral striatum) with connections from the striatum to the globus pallidus, substantia nigra, and projections from them to the specific thalamic nuclei, and, finally, they connect with the frontal lobe.7,8 They share the common structures, namely the frontal lobe, striatum, globus pallidus, substantia nigra, and the thalamus. Dysfunction of the fronto-subcortical circuitry could cause a wide range of behavioral changes, and the cingulate syndrome is characterized by AM, aspontaneity, and marked apathy.7
References 1. Auerbach S. Beitrag zur diagnostik der Geschwulste des Stirnhirn. Dtsch Z Nervenheilkd 1902;22:312-332. 2. Fisher CM. Abulia minor vs agitated behaviour. Clin Neurosurg 1983;3:319-331. 3. Damasio H, Damasio AR. Lesion analysis in neuropsychology. New York, NY: Oxford-University Press, 1989: 60-61. 4. Caplan LR, Schmchmann D, Kasi CS, et al. Caudate infarcts. Arch Neurol 1990;47:133-142. 5. Denny-Brown D. The basal ganglia and the relation to disorders of movement. New York, NY: Oxford University Press, 1962. 6. Segarra JM. Cerebral vascular disease and behaviour. I. Syndrome of the mescencephalic artery (basilar artery bifurcation). Arch Neurol 1970;22:408-418. 7. Cummnigs JL. Fronto-subcortical circuits and human behaviour. Arch Neurol 1993;50:873-880. 8. Alexander GE, Delaney MR, Strick PL. Parallel organisation of functionally segregated circuits linking basal ganglia and cortex. Ann Rev Neurosci 1990;9:357-381.