Pisa
Syndrome Without Neuroleptic Exposure in a Patient With Dementia of the Alzheimer Type
Shirish Patel,
MRCPsych;
Pierre N. Tariot, MD; Robert W. Hamill, MD
Abstract A case of Pisa syndrome in a 60-year-old man with dementia, probably of the Alzheimer type, who had never been exposed to any psychotropic medication is described. He also had some extrapyramidal features and myoclonus. Treatment with amantadine was slightly beneficial. We believe that this report is the first of its kind. ( J Geriatr Psychiatry Neurol
1991;4:48-51).
all used the
term Pisa syndrome to in which there is flexion posture of the body, neck, and head to one side accompanied by slight axial rotation of the trunk in the sagittal plane. Others have advocated the use of the term In pleurothotonus to describe the same their original report of three elderly females with
kbom
et
describe
syndrome.2
Ekbom et all attributed the synside effect of methylperone [a butyrophenone derivative]. Phenothiazines, both high and low potency, have been implicated subsequently. 2-1 Most cases of Pisa syndrome have been reported in patients with schizophrenia on neuroleptics, 2-7 and there have been a few reports of patients with presenilel and senile dementia of the Alzheimer type (DAT) developing the syndrome also in response to neuroleptics. It is believed to occur irrespective of the route of administration of the neuroleptics.’, 3-8 A case is presented here of a man whose postural abnormality fits exactly the description of Pisa syndrome by Ekbom et al.1 There is one significant difference, however: this patient had never been exposed to any kind of psychotropic medication prior to the onset of the syndrome.
presenile dementia, drome to
a
Received Oct 24, 1990. Received revised Dec 3, 1990. Acfor publication Jan 2, 1991. From the Departments of Psychiatry (Drs Patel and Tariot) and Neurology (Dr Hamill), Monroe Community Hospital, University of Rochester Medical Center, Rochester, NY. Address correspondence to Dr Patel, Psychiatry Unit, Monroe Community Hospital, 435 East Henrietta Road, Rochester, NY 14620.
cepted
Case
Report
a
.
-
..
Mr A.,
60-year-old, right-handed gentleman, was brought by his wife for evaluation of dementia of 7 years’ duration. Apart from a mild stutter since childhood, he had been healthy until age 53 years, when he presented to his family practitioner with a 10-month history of progressively increasing forgetfulness, difficulty in managing family finances, and a
lack of initiative. Over the next 18 months, his intellect, memory, and personality continued to decline steadily, and a clinical diagnosis of probable Alzheimer’s disease was made in accordance with the NIH Consensus Criteria.9 He retired because of this. Serial neuropsychological evaluations over the next 5 years confirmed the clinical impression of decline in his intellectual abilities, further supporting the diagnosis of DAT. These evaluations included tests measuring attention and concentration, verbal and nonverbal memory, language functions, visuospatial ability, and psychomotor speed. His performance showed progressive and significant impairments in all of these cognitive domains except for attention and concentration and visuospatial abilities, which were only mildly affected until the very late phase of his illness. In particular, there was a progressive deterioration in his ability to learn new material and retain. learned material. Over the same period of time, his Mini-Mental State Examination scorel° dropped from 25 to 7 of 30. Serial brain computed tomographic scans showed increasing cortical atrophy without other abnormalities, and an electroencepha-
48
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
logram performed at age 54 years was normal. Initial subsequent laboratory work-ups, including complete blood count; sedimentation rate; SMA-6 and SMA-12; thyroid function tests; serologic tests for syphilis, folate, vitamin 812; and urinalysis were all negative or normal. Mr A. had seen a neurologist at age 57 years for a second opinion on his dementing illness and for possible participation in an experimental drug study (for which he was found ineligible). Examination at the time revealed extrapyramidal features only, including bradykinesia, mild rigidity, and essential blepharospasm. Tremor was absent, and his gait was reported as stooped with normal arm swing. There was no family history of any neurodegenerative or psychiatric disorder, except for mild stuttering in a brother. There was no history of exposure to environmental or occupational toxin, loss of consciousness, head trauma, seizures, stroke, or other neurologic and
illness in his past. At the time of consultation (age 60 years) his wife reported new episodes during which he would lean to his left. This was particularly noticeable when he was seated but was also present when he was standing or walking. Initially, such episodes lasted for up to 3 days before he spontaneously recovered his normal erect posture and gait. After about 3 months, however, he began to adopt the leaning posture continuously. There was no antecedent change in his level of consciousness or any report of focal neurologic signs or symptoms. He had not experienced any falls or loss of balance and was on no medication. In particular, he had never been exposed to any psychotropic medication. Examination at this point showed his head to be tilted to his left, flexed toward his left shoulder. His neck was rigid, with a good passive range of lateral motion. His trunk was also curved to the left and rotated slightly backwards. With this posture he was still able to walk and run in a straight line with good-size steps. He was able to voluntarily straighten up and could move his head into a straight position but immediately returned to the previous posture. Frequent myoclonic jerking of all four limbs was also noted. Further neurologic examination revealed hypomimia and bilaterally equal brisk tendon reflexes in addition to the findings described earlier. Over the next 2 years, his posturing persisted and he became increasingly agitated, but no progression of his parkinsonian features or myoclonic jerking was noted. In an attempt to control the
former, levodopa-carbidopa and amantadine
were
tried in succession. Treatment with the latter improved his posture slightly, but it had to be discontinued because it worsened his agitation. About 14 months after he first started leaning to his left, he was prescribed haloperidol to control his agitation and violent behavior. This, too, had to be discontinued after an oculogyric crisis. There was no change in the degree of his posturing or the appearance of any new dystonic symptoms while he was on halo-
peridol. At this point (age 62 years), the patient is unable feed or dress himself and is virtually mute. He continues to maintain the left-sided leaning posture.
to
Discussion The case described above is one of dementia, probably of the Alzheimer type, with parkinsonian features ; the postural disturbance is identical to the description of Pisa syndrome by Ekbom et al.1 Once his posture became persistent, it was maintained both at rest and during activities like walking and running. This report is believed to be unique, as an extensive review of the literature failed to disclose a similar case of Pisa syndrome in a patient with DAT, or indeed a patient with any other diagnosis, who had not been exposed to neuroleptics. We believe this case contributes to the fund of clinical knowledge about the phenomenology of DAT. The patients’ extrapyramidal features are not inconsistent with the clinical diagnosis, since they are 11’12 In common in patients with Alzheimer’s disease. their systematic study of extrapyramidal signs in patients with Alzheimer’s disease who had not been treated with neuroleptics or dopamine-depleting drugs, Molsa et al 12 found that hypokinesia, hypomimia, rigidity, and flexion posture are all very common, while resting tremor was rare. Myoclonic jerking of extremities, though infrequent, is also known to occur in Alzheimer’s disease. 13 Mayeux et al 14 have described a group of patients with DAT with myoclonus who had younger age at onset, rapidly progressive illness, and mutism. Mr A.’s age at onset of dementia was 53 years, and his intellectual decline was severe, leaving him virtually mute. All these findings are consistent with the diagnosis of DAT and, together with the absence of other typical features of Parkinson’s disease, make it unlikely that his postural abnormality was a result of Parkinson’s disease. Although the patient’s cognitive disorder was the primary clinical feature early in the course of his
49
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
illness and led to the
diagnosis of DAT, the evolving extrapyramidal signs made us consider other basal ganglia syndromes associated with dementia. The two major diagnostic possibilities considered were progressive supranuclear palsy and striatonigral degeneration. Absence of classic ophthalmoplegia 15 and atypical signs 16 make the diagnosis of progressive supranuclear palsy highly unlikely, while the patient’s early and progressive dementia was atypical for both illnesses. 17 A Lewy body variant of Alzheimer’s disease’8
was also considered. In the absence of severely impaired attention or visuospatial ability on neuropsychological testing and of masked facies or tremor on neurologic examination, this diagnostic possibility appears unlikely. A postmortem examination of the brain, however, would help clarify the issue. Pisa syndrome has been considered a form of tardive dystonia.3 Burke et a119 used the following criteria to diagnose tardive dystonia: (1) the presence of chronic dystonia; (2) a history of antipsychotic drug treatment preceding or concurrent with the onset of dystonia; (3) exclusion of known cases of secondary dystonia by appropriate clinical and laboratory evaluation; and (4) a negative family history for dystonia. The case presented here fulfills all these criteria with the exception of the second criterion. He had not received any antipsychotic medication before or at the time he developed the postural abnormality. Except for essential blepharospasm he had no other dystonic symptoms. In particular, there was no evidence of tardive dyskinesia, which has been reported to coexist with tardive dystonia. 1,3,20-22 The etiology and pathophysiology of Pisa syndrome are uncertain. Postulations include the role of dopaminergic and cholinergic,19 noradrenergic and serotonergic,23 and dopamine-acetylcholine systems.~ It should be noted, however, that all of these reports pertain to neuroleptic-induced Pisa syndrome. This case report may enable the above hypotheses to be reconsidered and suggests in particular that Pisa syndrome may be a syndrome with multiple etiologies. The treatment of Pisa syndrome is not well established.24,25 Where it has been neuroleptic induced, withdrawal of the same has been shown to be helpful. 1,3,7,19 Other pharmacologic strategies used with varying degrees of success include antitetrabenazine, 19,26 carbamazcholinergic and epine, propranolol clonazepam,19 and amantadine.8 Electroconvulsive therapy has been reported to bring about improvement in some cases. 25,27 In the case reported above, some improvement
drugs/,19
in the posture resulted after amantadine was prescribed. Unfortunately, a lengthy trial could not be given because the drug had to be discontinued after the patient became very agitated. Levodopacarbidopa, however, was of no benefit in this case.
Acknowledgments These efforts were supported in part by grants from the National Institute of Mental Health (5 K07 MH00733 02; 2 P50 MH40381 04) and the National Institute of Aging (AI 03644). The authors would like to thank Maureen E. Herbstsommer for her secretarial assistance.
References 1. Ekbom K, Lindhom H, Ljungberg L: New dystonic syndrome associated with butyrophenone therapy. Z Neurol 1972;202: 94-103. 2. Pilette WL: Pisa syndrome, or pleurothotonus, letter. Am J
Psychiatry 1987;144:969-970. syndrome:
3. Yassa R: The Pisa 4.
5.
A
report of
two cases.
Br
J Psy-
chiatry 1985;146:93-95. Saxena S: Tardive dystonia and Pisa syndrome, letter. Br J Psychiatry 1986;149:524. Guy N, Raps A, Assael M: The Pisa syndrome during maintenance antipsychotic therapy, letter. Am J Psychiatry 1986;
143:1492. 6. Chui HFK, Li SW, Lee S: Pisa syndrome in a Chinese patient. Aust N Z J Psychiatry 1989;23:127-128. 7. Amore M, Cerisoli M, Campanile S, et al: Pisa syndrome: Report of a case. ItalJ Neurol Sci 1988;9:273-274. 8. Davidson M, Powchik P, Davis KL: Pisa syndrome in Alzheimer’s disease. Biol Psychiatry 1988;23:209-214. 9. McKhann G, Drachman D, Folstein M, et al: Clinical diagnosis of Alzheimer’s disease: Report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Services task force on Alzheimer’s disease. Neurology
1984;34:939-944. 10. Folstein MR, Folstein SE, McHugh PR: Mini-Mental State: A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198. 11. Pearce J: The extrapyramidal disorder of Alzheimer’s disease.
Eur 1974;12:94-103. Neurol PK, Martilla RJ, Rinne UK: Extrapyramidal signs
12. Molsa
in
Alzheimer’s disease. Neurology 1984;34:1114-1116. 13. Faden AI, Townsend JJ: Myoclonus in Alzheimer’s disease. 14.
Arch Neurol 1976;33:278-280. Mayeux R, Stern Y, Spanton S: Heterogeneity in dementia of the Alzheimer type: Evidence of subgroups. Neurology
1985;35:453-461. 15. Steele JC, Richardson JC, Olszewski J: Progressive supranuclear palsy: A heterogenous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-359. 16. Davis PH, Bergeron C, McLachlan DR: Atypical presentation of progressive supranuclear palsy. Ann Neurol 1985 ;17 :337343. 17. Adams RD, Bugaert L van, van der Eecken H: Striato-nigral degeneration. J Neuropathol Exp Neurol 1964;23:584-608.
50
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
18. Hansen L, Salmon D, Galasko D, et al: The Lewy body variant of Alzheimer’s disease: A clinical and pathological entity.
Neurology 1990;40:1-8. 19. Burke RE, Fahn S, Jankovic J, et al: Tardive dystonia: Lateonset and persistent dystonia caused by antipsychotic drugs. 20. 21.
22. 23.
Neurology 1982;32:1335-1346. Nasrallah HA, Pappas NJ, Crowe RR: Oculogyric dystonia in tardive dyskinesia. Am J Psychiatry 1980;137:850-851. Gardos G: Dystonic reactions during maintenance antipsychotic therapy. Am J Psychiatry 1981;138:114-115. Munetz MR: Oculogyric crises and tardive dyskinesia. AmJ Psychiatry 1980;137:1628. Remington GJ: The Pisa syndrome: Possible role for serotonin
and noradrenaline, letter. J Clin 229. 24. Burke RE, Fahn
appropriate
S, Jankovic J,
use
of
Psychopharmacol 1988 ;8 :228-
et al: Tardive
neuroleptic drugs,
dystonia letter.
and inLancet
1982;1:1299. 25. Kwentus JA, Schulz SC, Hart RP: Tardive dystonia, catatonia and electroconvulsive therapy. J Nerv Ment Dis 1984;172: 171-173. 26. Kang UJ, Burke RE, Fahn S: Natural history and treatment of tardive dystonia. Mov Disord 1986;1:193-208. 27. Adityanjee, Jayaswal SK, Chan TM, et al: Temporary remission of tardive dystonia following electroconvulsive therapy. Br J Psychiatry 1990;156:433-435.
Continued from page 2 Since there is little in the
literature on obsessive-compulsive disorders, I have written a relatively brief review of this topic, which is included as a topical review article in this issue. Even though this disorder rarely begins in old age, many elderly patients have suffered from this disorder for decades. Due to recent publicity about this disorder, elderly patients are seeking physicians for evaluation and treatment. Specific issues concerning treatment of elderly patients with this disorder are reviewed. We have four enlightening case reports this month. Dr Draupathi Nambudiri and colleagues of Cornell University Medical College report three case histories which illustrate their hypothesis that lithium-induced neurotoxicity can take much longer to resolve than 2 to 3 weeks as previously reported. In addition, their cases illustrate that reintroduction of lithium can be a safe and therapeutically effective alternative to anticonvulsant medications in elderly patients who previously suffered toxic central nervous system reactions. Drs Kelly Botteron and associates of the Psychiatry Department of Washington University School of Medicine described three patients with late-onset psychosis who were treated with electroconvulsive therapy and report that preexisting structural brain changes predict a poor response to this treatment. In addition, consistent with previous reports, the presence of caudate hy-
geriatric
perintensities on magnetic resonance imaging (MRI) predicted prolonged electroconvulsive therapyinduced delirium. Dr Sandra Swantek et al from St Louis University School of Medicine and the University of Washington discuss the successful use of carbamazepine (Tegretol) as a treatment for benzodiazepine withdrawal in four elderly patients. The authors suggest that this treatment regimen take place in a hospital setting where close monitoring can occur.
The last case report is by Drs Shirish Patel and colleagues from the Departments of Psychiatry and Neurology at the University of Rochester Medical Center. These authors describe a case of Pisa syndrome (ie, flexion of the body, neck, and head to one side accompanied by slight axial rotation of the trunk in the sagittal plane) in an elderly man with dementia, never having been exposed to any psychotropic medication, who responded somewhat to amantadine. Once again, I have enjoyed reading these papers, and I hope that you will also. Thank you once again for your support. Please write with suggestions and, as always, we welcome Letters to the Editor that comment on any of the papers presented. Best wishes!
MAJ
51
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Syndrome Without Neuroleptic Exposure in a Patient With Dementia of the Alzheimer Type
Shirish Patel,
MRCPsych;
Pierre N. Tariot, MD; Robert W. Hamill, MD
Abstract A case of Pisa syndrome in a 60-year-old man with dementia, probably of the Alzheimer type, who had never been exposed to any psychotropic medication is described. He also had some extrapyramidal features and myoclonus. Treatment with amantadine was slightly beneficial. We believe that this report is the first of its kind. ( J Geriatr Psychiatry Neurol
1991;4:48-51).
all used the
term Pisa syndrome to in which there is flexion posture of the body, neck, and head to one side accompanied by slight axial rotation of the trunk in the sagittal plane. Others have advocated the use of the term In pleurothotonus to describe the same their original report of three elderly females with
kbom
et
describe
syndrome.2
Ekbom et all attributed the synside effect of methylperone [a butyrophenone derivative]. Phenothiazines, both high and low potency, have been implicated subsequently. 2-1 Most cases of Pisa syndrome have been reported in patients with schizophrenia on neuroleptics, 2-7 and there have been a few reports of patients with presenilel and senile dementia of the Alzheimer type (DAT) developing the syndrome also in response to neuroleptics. It is believed to occur irrespective of the route of administration of the neuroleptics.’, 3-8 A case is presented here of a man whose postural abnormality fits exactly the description of Pisa syndrome by Ekbom et al.1 There is one significant difference, however: this patient had never been exposed to any kind of psychotropic medication prior to the onset of the syndrome.
presenile dementia, drome to
a
Received Oct 24, 1990. Received revised Dec 3, 1990. Acfor publication Jan 2, 1991. From the Departments of Psychiatry (Drs Patel and Tariot) and Neurology (Dr Hamill), Monroe Community Hospital, University of Rochester Medical Center, Rochester, NY. Address correspondence to Dr Patel, Psychiatry Unit, Monroe Community Hospital, 435 East Henrietta Road, Rochester, NY 14620.
cepted
Case
Report
a
.
-
..
Mr A.,
60-year-old, right-handed gentleman, was brought by his wife for evaluation of dementia of 7 years’ duration. Apart from a mild stutter since childhood, he had been healthy until age 53 years, when he presented to his family practitioner with a 10-month history of progressively increasing forgetfulness, difficulty in managing family finances, and a
lack of initiative. Over the next 18 months, his intellect, memory, and personality continued to decline steadily, and a clinical diagnosis of probable Alzheimer’s disease was made in accordance with the NIH Consensus Criteria.9 He retired because of this. Serial neuropsychological evaluations over the next 5 years confirmed the clinical impression of decline in his intellectual abilities, further supporting the diagnosis of DAT. These evaluations included tests measuring attention and concentration, verbal and nonverbal memory, language functions, visuospatial ability, and psychomotor speed. His performance showed progressive and significant impairments in all of these cognitive domains except for attention and concentration and visuospatial abilities, which were only mildly affected until the very late phase of his illness. In particular, there was a progressive deterioration in his ability to learn new material and retain. learned material. Over the same period of time, his Mini-Mental State Examination scorel° dropped from 25 to 7 of 30. Serial brain computed tomographic scans showed increasing cortical atrophy without other abnormalities, and an electroencepha-
48
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
logram performed at age 54 years was normal. Initial subsequent laboratory work-ups, including complete blood count; sedimentation rate; SMA-6 and SMA-12; thyroid function tests; serologic tests for syphilis, folate, vitamin 812; and urinalysis were all negative or normal. Mr A. had seen a neurologist at age 57 years for a second opinion on his dementing illness and for possible participation in an experimental drug study (for which he was found ineligible). Examination at the time revealed extrapyramidal features only, including bradykinesia, mild rigidity, and essential blepharospasm. Tremor was absent, and his gait was reported as stooped with normal arm swing. There was no family history of any neurodegenerative or psychiatric disorder, except for mild stuttering in a brother. There was no history of exposure to environmental or occupational toxin, loss of consciousness, head trauma, seizures, stroke, or other neurologic and
illness in his past. At the time of consultation (age 60 years) his wife reported new episodes during which he would lean to his left. This was particularly noticeable when he was seated but was also present when he was standing or walking. Initially, such episodes lasted for up to 3 days before he spontaneously recovered his normal erect posture and gait. After about 3 months, however, he began to adopt the leaning posture continuously. There was no antecedent change in his level of consciousness or any report of focal neurologic signs or symptoms. He had not experienced any falls or loss of balance and was on no medication. In particular, he had never been exposed to any psychotropic medication. Examination at this point showed his head to be tilted to his left, flexed toward his left shoulder. His neck was rigid, with a good passive range of lateral motion. His trunk was also curved to the left and rotated slightly backwards. With this posture he was still able to walk and run in a straight line with good-size steps. He was able to voluntarily straighten up and could move his head into a straight position but immediately returned to the previous posture. Frequent myoclonic jerking of all four limbs was also noted. Further neurologic examination revealed hypomimia and bilaterally equal brisk tendon reflexes in addition to the findings described earlier. Over the next 2 years, his posturing persisted and he became increasingly agitated, but no progression of his parkinsonian features or myoclonic jerking was noted. In an attempt to control the
former, levodopa-carbidopa and amantadine
were
tried in succession. Treatment with the latter improved his posture slightly, but it had to be discontinued because it worsened his agitation. About 14 months after he first started leaning to his left, he was prescribed haloperidol to control his agitation and violent behavior. This, too, had to be discontinued after an oculogyric crisis. There was no change in the degree of his posturing or the appearance of any new dystonic symptoms while he was on halo-
peridol. At this point (age 62 years), the patient is unable feed or dress himself and is virtually mute. He continues to maintain the left-sided leaning posture.
to
Discussion The case described above is one of dementia, probably of the Alzheimer type, with parkinsonian features ; the postural disturbance is identical to the description of Pisa syndrome by Ekbom et al.1 Once his posture became persistent, it was maintained both at rest and during activities like walking and running. This report is believed to be unique, as an extensive review of the literature failed to disclose a similar case of Pisa syndrome in a patient with DAT, or indeed a patient with any other diagnosis, who had not been exposed to neuroleptics. We believe this case contributes to the fund of clinical knowledge about the phenomenology of DAT. The patients’ extrapyramidal features are not inconsistent with the clinical diagnosis, since they are 11’12 In common in patients with Alzheimer’s disease. their systematic study of extrapyramidal signs in patients with Alzheimer’s disease who had not been treated with neuroleptics or dopamine-depleting drugs, Molsa et al 12 found that hypokinesia, hypomimia, rigidity, and flexion posture are all very common, while resting tremor was rare. Myoclonic jerking of extremities, though infrequent, is also known to occur in Alzheimer’s disease. 13 Mayeux et al 14 have described a group of patients with DAT with myoclonus who had younger age at onset, rapidly progressive illness, and mutism. Mr A.’s age at onset of dementia was 53 years, and his intellectual decline was severe, leaving him virtually mute. All these findings are consistent with the diagnosis of DAT and, together with the absence of other typical features of Parkinson’s disease, make it unlikely that his postural abnormality was a result of Parkinson’s disease. Although the patient’s cognitive disorder was the primary clinical feature early in the course of his
49
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
illness and led to the
diagnosis of DAT, the evolving extrapyramidal signs made us consider other basal ganglia syndromes associated with dementia. The two major diagnostic possibilities considered were progressive supranuclear palsy and striatonigral degeneration. Absence of classic ophthalmoplegia 15 and atypical signs 16 make the diagnosis of progressive supranuclear palsy highly unlikely, while the patient’s early and progressive dementia was atypical for both illnesses. 17 A Lewy body variant of Alzheimer’s disease’8
was also considered. In the absence of severely impaired attention or visuospatial ability on neuropsychological testing and of masked facies or tremor on neurologic examination, this diagnostic possibility appears unlikely. A postmortem examination of the brain, however, would help clarify the issue. Pisa syndrome has been considered a form of tardive dystonia.3 Burke et a119 used the following criteria to diagnose tardive dystonia: (1) the presence of chronic dystonia; (2) a history of antipsychotic drug treatment preceding or concurrent with the onset of dystonia; (3) exclusion of known cases of secondary dystonia by appropriate clinical and laboratory evaluation; and (4) a negative family history for dystonia. The case presented here fulfills all these criteria with the exception of the second criterion. He had not received any antipsychotic medication before or at the time he developed the postural abnormality. Except for essential blepharospasm he had no other dystonic symptoms. In particular, there was no evidence of tardive dyskinesia, which has been reported to coexist with tardive dystonia. 1,3,20-22 The etiology and pathophysiology of Pisa syndrome are uncertain. Postulations include the role of dopaminergic and cholinergic,19 noradrenergic and serotonergic,23 and dopamine-acetylcholine systems.~ It should be noted, however, that all of these reports pertain to neuroleptic-induced Pisa syndrome. This case report may enable the above hypotheses to be reconsidered and suggests in particular that Pisa syndrome may be a syndrome with multiple etiologies. The treatment of Pisa syndrome is not well established.24,25 Where it has been neuroleptic induced, withdrawal of the same has been shown to be helpful. 1,3,7,19 Other pharmacologic strategies used with varying degrees of success include antitetrabenazine, 19,26 carbamazcholinergic and epine, propranolol clonazepam,19 and amantadine.8 Electroconvulsive therapy has been reported to bring about improvement in some cases. 25,27 In the case reported above, some improvement
drugs/,19
in the posture resulted after amantadine was prescribed. Unfortunately, a lengthy trial could not be given because the drug had to be discontinued after the patient became very agitated. Levodopacarbidopa, however, was of no benefit in this case.
Acknowledgments These efforts were supported in part by grants from the National Institute of Mental Health (5 K07 MH00733 02; 2 P50 MH40381 04) and the National Institute of Aging (AI 03644). The authors would like to thank Maureen E. Herbstsommer for her secretarial assistance.
References 1. Ekbom K, Lindhom H, Ljungberg L: New dystonic syndrome associated with butyrophenone therapy. Z Neurol 1972;202: 94-103. 2. Pilette WL: Pisa syndrome, or pleurothotonus, letter. Am J
Psychiatry 1987;144:969-970. syndrome:
3. Yassa R: The Pisa 4.
5.
A
report of
two cases.
Br
J Psy-
chiatry 1985;146:93-95. Saxena S: Tardive dystonia and Pisa syndrome, letter. Br J Psychiatry 1986;149:524. Guy N, Raps A, Assael M: The Pisa syndrome during maintenance antipsychotic therapy, letter. Am J Psychiatry 1986;
143:1492. 6. Chui HFK, Li SW, Lee S: Pisa syndrome in a Chinese patient. Aust N Z J Psychiatry 1989;23:127-128. 7. Amore M, Cerisoli M, Campanile S, et al: Pisa syndrome: Report of a case. ItalJ Neurol Sci 1988;9:273-274. 8. Davidson M, Powchik P, Davis KL: Pisa syndrome in Alzheimer’s disease. Biol Psychiatry 1988;23:209-214. 9. McKhann G, Drachman D, Folstein M, et al: Clinical diagnosis of Alzheimer’s disease: Report of the NINCDS-ADRDA work group under the auspices of Department of Health and Human Services task force on Alzheimer’s disease. Neurology
1984;34:939-944. 10. Folstein MR, Folstein SE, McHugh PR: Mini-Mental State: A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189-198. 11. Pearce J: The extrapyramidal disorder of Alzheimer’s disease.
Eur 1974;12:94-103. Neurol PK, Martilla RJ, Rinne UK: Extrapyramidal signs
12. Molsa
in
Alzheimer’s disease. Neurology 1984;34:1114-1116. 13. Faden AI, Townsend JJ: Myoclonus in Alzheimer’s disease. 14.
Arch Neurol 1976;33:278-280. Mayeux R, Stern Y, Spanton S: Heterogeneity in dementia of the Alzheimer type: Evidence of subgroups. Neurology
1985;35:453-461. 15. Steele JC, Richardson JC, Olszewski J: Progressive supranuclear palsy: A heterogenous degeneration involving the brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-359. 16. Davis PH, Bergeron C, McLachlan DR: Atypical presentation of progressive supranuclear palsy. Ann Neurol 1985 ;17 :337343. 17. Adams RD, Bugaert L van, van der Eecken H: Striato-nigral degeneration. J Neuropathol Exp Neurol 1964;23:584-608.
50
Downloaded from jgp.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 31, 2015
18. Hansen L, Salmon D, Galasko D, et al: The Lewy body variant of Alzheimer’s disease: A clinical and pathological entity.
Neurology 1990;40:1-8. 19. Burke RE, Fahn S, Jankovic J, et al: Tardive dystonia: Lateonset and persistent dystonia caused by antipsychotic drugs. 20. 21.
22. 23.
Neurology 1982;32:1335-1346. Nasrallah HA, Pappas NJ, Crowe RR: Oculogyric dystonia in tardive dyskinesia. Am J Psychiatry 1980;137:850-851. Gardos G: Dystonic reactions during maintenance antipsychotic therapy. Am J Psychiatry 1981;138:114-115. Munetz MR: Oculogyric crises and tardive dyskinesia. AmJ Psychiatry 1980;137:1628. Remington GJ: The Pisa syndrome: Possible role for serotonin
and noradrenaline, letter. J Clin 229. 24. Burke RE, Fahn
appropriate
S, Jankovic J,
use
of
Psychopharmacol 1988 ;8 :228-
et al: Tardive
neuroleptic drugs,
dystonia letter.
and inLancet
1982;1:1299. 25. Kwentus JA, Schulz SC, Hart RP: Tardive dystonia, catatonia and electroconvulsive therapy. J Nerv Ment Dis 1984;172: 171-173. 26. Kang UJ, Burke RE, Fahn S: Natural history and treatment of tardive dystonia. Mov Disord 1986;1:193-208. 27. Adityanjee, Jayaswal SK, Chan TM, et al: Temporary remission of tardive dystonia following electroconvulsive therapy. Br J Psychiatry 1990;156:433-435.
Continued from page 2 Since there is little in the
literature on obsessive-compulsive disorders, I have written a relatively brief review of this topic, which is included as a topical review article in this issue. Even though this disorder rarely begins in old age, many elderly patients have suffered from this disorder for decades. Due to recent publicity about this disorder, elderly patients are seeking physicians for evaluation and treatment. Specific issues concerning treatment of elderly patients with this disorder are reviewed. We have four enlightening case reports this month. Dr Draupathi Nambudiri and colleagues of Cornell University Medical College report three case histories which illustrate their hypothesis that lithium-induced neurotoxicity can take much longer to resolve than 2 to 3 weeks as previously reported. In addition, their cases illustrate that reintroduction of lithium can be a safe and therapeutically effective alternative to anticonvulsant medications in elderly patients who previously suffered toxic central nervous system reactions. Drs Kelly Botteron and associates of the Psychiatry Department of Washington University School of Medicine described three patients with late-onset psychosis who were treated with electroconvulsive therapy and report that preexisting structural brain changes predict a poor response to this treatment. In addition, consistent with previous reports, the presence of caudate hy-
geriatric
perintensities on magnetic resonance imaging (MRI) predicted prolonged electroconvulsive therapyinduced delirium. Dr Sandra Swantek et al from St Louis University School of Medicine and the University of Washington discuss the successful use of carbamazepine (Tegretol) as a treatment for benzodiazepine withdrawal in four elderly patients. The authors suggest that this treatment regimen take place in a hospital setting where close monitoring can occur.
The last case report is by Drs Shirish Patel and colleagues from the Departments of Psychiatry and Neurology at the University of Rochester Medical Center. These authors describe a case of Pisa syndrome (ie, flexion of the body, neck, and head to one side accompanied by slight axial rotation of the trunk in the sagittal plane) in an elderly man with dementia, never having been exposed to any psychotropic medication, who responded somewhat to amantadine. Once again, I have enjoyed reading these papers, and I hope that you will also. Thank you once again for your support. Please write with suggestions and, as always, we welcome Letters to the Editor that comment on any of the papers presented. Best wishes!
MAJ
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