1034 TEMPORAL-LOBE EPILEPSY PRESENTING AS FUGUE STATE can present in a variety of We have seen an unusual example of this. A 42-year-old senior executive was admitted with the complaint of amnesia for the previous four days, during which he apparently signed and cashed substantial cheques belonging to his company. On mental-status examination he showed no abnormality; he looked concerned and seemed prepared for legal consequences. He reported having several similar episodes in the past 20 years, all of which had been precipitated by pressure at work, each lasting 3-5 days. On some of these occasions he might have flown to Las Vegas, presumably to gamble, but neither he nor his family were able to give any account of these days. He had also had short amnestic epi-
SIR,—Temporal-lobe epilepsy
forms of fugue
state.
most preceded by acute feelings of panic accompanied by a feeling of warmth crawling up from his stomach. Because he was afraid his mind "was playing tricks on him", he learned to dissemble very skilfully. His wife had experience of this several times after his longer trips. She also noticed that a few hours before her husband’s absence, he would answer her questions absentmindedly and he would drive a car in an automatic fashion. On returning, he would always fall into a very deep sleep lasting up to 12 h. An E.E.G. taken on the day of admission revealed clear-cut, sharp wave complexes arising from the left temporal region and occasionally on the right side. An E.E.G. with sphenoidal
sodes,
electrodes, done a week later, was normal. On neurological examination mild weakness on the right side was noticed. Computerised tomography showed an area of increased density along the anterior inferior aspect of the temporal lobe, but this was felt to represent an artifact. Carotid arteriography was normal. The patient was discharged on phenytoin with a diagnosis of temporal-lobe fnilfrxv
Department of Psychiatry, School of Medicine, University of Alabama in Birmingham, Birmingham, Alabama 35294, U.S.A.
VERA PRCHAL JOHN R. SMYTHIES
"OFF PERIOD" DYSTONIA AND "ON PERIOD" CHOREOATHETOSIS IN LEVODOPA-TREATED PATIENTS WITH PARKINSON’S DISEASE
SIR,-Involuntary movements and disabling oscillations in performance ("on-off" effects) are the two main complications of long-term levodopa treatment in Parkinson’s disease. In 92 of our patients treated for longer than 6 years, 59 (65%) have disabling dyskinesias and 45(49%) suffer from "on-off" effects. When the two complications occur together, involuntary move’ments usually happen during the "on" periods of relative mobility and are most frequently choreic or athetoid in type. Dose-dependent peak-dose dyskinesias are commonest, starting 30 min to 2 h after each dose of levodopa and lasting for 15 min to 4 h.1 The only effective remedy is reducing the dose of levodopa, inevitably at the expense of increased parkinsonian disability. The other well-recognised pattern is the longduration dystonia-improvement-dystonia sequence2of which biphasic (onset and end-of-dose) dyskinesias are an integral part.3, 4 We describe here involuntary movements during "off ’ periods, particularly in association with early morning akinesia. The movements are dystonic and may be generalised, although they are usually confined to the legs. A 61-year-old woman with a 5-year history of predoYahr, M. D., Duvoisin, R. C., Shear, M. J., Barrett, R. E., Hoehn, M. M. Archs Neurol 1969, 21, 343. 2. Muenter, M., Sharpless, N. S., Tyce, G. M., Darley, F. L. Mayo Clin. Proc. 1977, 52, 163. 3. Barbeau, A. Lancet, 1975, i, 756. 4. L’Hermitte, F., Agid, Y., Signoret, J.-L., Studler, J.-M. Rev. Neurol. 1977, 133, 297. 1.
minantly right-sided idiopathic disease began to complain of generalised cramps after 3 years of levodopa treatment. Strong extension movements of her legs and sustained, painful dystonia of her right ankle and foot woke her each morning. Her jaw deviated to the right preventing mouth-opening, her neck was forcibly extended, and her right shoulder was elevated and internally rotated with flexion at the elbow. This was accompanied by profuse, generalised sweating and dilatation of the pupils. About 30 min after the first levodopa dose of the morning, her dystonic symptoms subsided, and were followed by a gradual onset of choreoathetosis. Less severe dystonia recurred in the early afternoon, again with severe akinesia. We have seen this sequence in 9 of 120 patients treated for 3 years or more with levodopa, none of whom had dystonia at diagnosis. All of them had severe "on-off" disabilities and the mean time of onset was 3. 8 years after beginning treatment. The severity and duration of dystonia may be reduced by increasing the dose of levodopa or by adding bromocriptine. (-)-deprenyl, which improves early morning akinesia, is ineffective. These findings and the description of distinct choreic and torsion varieties of levodopa-induced voluntary movements’ suggest separate biochemical mechanisms. The recent demonstration of two separate types of dopaminergic neurons which mediate inhibition and excitation in animals6 might offer a possible explanation. Further studies of levodopainduced abnormal movements may throw light on the pathogenesis of dystonia and chorea. A. J. LEES Department of Neurology, K. M. SHAW University College Hospital, G. M. STERN London WC1
IMPROVEMENT OF SPINAL AMYOTROPHY BY PENICILLAMINE THERAPY seen an impressive and sustained improvein two patients with rapidly progressive spinal amyotrophy who were given penicillamine. In August, 1976, a woman, now aged 21, had difficulty in climbing stairs. A month later combing her hair became a problem. By November she could not squat, rise, or walk on heels or toes without support. In February, 1977, she could not sit up from a flat supine position without help. The muscles of both limb girdles were atrophied. All deep reflexes were absent. The first electromyogram (E.M.G.) (November, 1976) pointed to motor-neurone loss with wide potentials exhibiting clear central driving, especially in deltoid muscles. Motor and sensory conduction velocities were normal. In February, 1977, the motor-neurone loss in deltoid muscle was extreme. Cerebrospinal fluid and plasma creatine kinase, alkaline phosphatase, and transaminases levels were normal. No signs of inflammatory or immunological disturbance were seen. The patient had been working for two years as a typesetter on a modern machine. Because of the remote possibility of lead intoxication the patient was tested for lead toxicity but nothing abnormal was found. To mobilise any stored lead in bones the patient was given penicillamine 300 mg three times a day for 5 days. Some slight improvement of muscle strength was noted by the fifth day. On March 6 continuous therapy with penicillamine was started (3 x 300 mg/day) and the patient is still on this regimen. After a month of this therapy the patient could walk on her heels or toes, raise her arms above her head, comb her hair, get up from a supine position without support, and lift a weight of about 9 kg. 2 months later she could squat and rise without help, carry a small child for an hour, walk for many hours, and do heavy agricultural work. In the E.M.G. (deltoid) besides the single-oscillation pattern of big potentials several polyphasic low-voltage potentials with
SIR,—We have
ment
5. Parkes, J. D., Bedard, P., Marsden, C. D. Lancet, 1976, ii, 155. 6. Cools, A. R., van Rossum, J. M. Psychopharmacologia, 1976, 45,
243.
SIR,—Temporal-lobe epilepsy
forms of fugue
state.
most preceded by acute feelings of panic accompanied by a feeling of warmth crawling up from his stomach. Because he was afraid his mind "was playing tricks on him", he learned to dissemble very skilfully. His wife had experience of this several times after his longer trips. She also noticed that a few hours before her husband’s absence, he would answer her questions absentmindedly and he would drive a car in an automatic fashion. On returning, he would always fall into a very deep sleep lasting up to 12 h. An E.E.G. taken on the day of admission revealed clear-cut, sharp wave complexes arising from the left temporal region and occasionally on the right side. An E.E.G. with sphenoidal
sodes,
electrodes, done a week later, was normal. On neurological examination mild weakness on the right side was noticed. Computerised tomography showed an area of increased density along the anterior inferior aspect of the temporal lobe, but this was felt to represent an artifact. Carotid arteriography was normal. The patient was discharged on phenytoin with a diagnosis of temporal-lobe fnilfrxv
Department of Psychiatry, School of Medicine, University of Alabama in Birmingham, Birmingham, Alabama 35294, U.S.A.
VERA PRCHAL JOHN R. SMYTHIES
"OFF PERIOD" DYSTONIA AND "ON PERIOD" CHOREOATHETOSIS IN LEVODOPA-TREATED PATIENTS WITH PARKINSON’S DISEASE
SIR,-Involuntary movements and disabling oscillations in performance ("on-off" effects) are the two main complications of long-term levodopa treatment in Parkinson’s disease. In 92 of our patients treated for longer than 6 years, 59 (65%) have disabling dyskinesias and 45(49%) suffer from "on-off" effects. When the two complications occur together, involuntary move’ments usually happen during the "on" periods of relative mobility and are most frequently choreic or athetoid in type. Dose-dependent peak-dose dyskinesias are commonest, starting 30 min to 2 h after each dose of levodopa and lasting for 15 min to 4 h.1 The only effective remedy is reducing the dose of levodopa, inevitably at the expense of increased parkinsonian disability. The other well-recognised pattern is the longduration dystonia-improvement-dystonia sequence2of which biphasic (onset and end-of-dose) dyskinesias are an integral part.3, 4 We describe here involuntary movements during "off ’ periods, particularly in association with early morning akinesia. The movements are dystonic and may be generalised, although they are usually confined to the legs. A 61-year-old woman with a 5-year history of predoYahr, M. D., Duvoisin, R. C., Shear, M. J., Barrett, R. E., Hoehn, M. M. Archs Neurol 1969, 21, 343. 2. Muenter, M., Sharpless, N. S., Tyce, G. M., Darley, F. L. Mayo Clin. Proc. 1977, 52, 163. 3. Barbeau, A. Lancet, 1975, i, 756. 4. L’Hermitte, F., Agid, Y., Signoret, J.-L., Studler, J.-M. Rev. Neurol. 1977, 133, 297. 1.
minantly right-sided idiopathic disease began to complain of generalised cramps after 3 years of levodopa treatment. Strong extension movements of her legs and sustained, painful dystonia of her right ankle and foot woke her each morning. Her jaw deviated to the right preventing mouth-opening, her neck was forcibly extended, and her right shoulder was elevated and internally rotated with flexion at the elbow. This was accompanied by profuse, generalised sweating and dilatation of the pupils. About 30 min after the first levodopa dose of the morning, her dystonic symptoms subsided, and were followed by a gradual onset of choreoathetosis. Less severe dystonia recurred in the early afternoon, again with severe akinesia. We have seen this sequence in 9 of 120 patients treated for 3 years or more with levodopa, none of whom had dystonia at diagnosis. All of them had severe "on-off" disabilities and the mean time of onset was 3. 8 years after beginning treatment. The severity and duration of dystonia may be reduced by increasing the dose of levodopa or by adding bromocriptine. (-)-deprenyl, which improves early morning akinesia, is ineffective. These findings and the description of distinct choreic and torsion varieties of levodopa-induced voluntary movements’ suggest separate biochemical mechanisms. The recent demonstration of two separate types of dopaminergic neurons which mediate inhibition and excitation in animals6 might offer a possible explanation. Further studies of levodopainduced abnormal movements may throw light on the pathogenesis of dystonia and chorea. A. J. LEES Department of Neurology, K. M. SHAW University College Hospital, G. M. STERN London WC1
IMPROVEMENT OF SPINAL AMYOTROPHY BY PENICILLAMINE THERAPY seen an impressive and sustained improvein two patients with rapidly progressive spinal amyotrophy who were given penicillamine. In August, 1976, a woman, now aged 21, had difficulty in climbing stairs. A month later combing her hair became a problem. By November she could not squat, rise, or walk on heels or toes without support. In February, 1977, she could not sit up from a flat supine position without help. The muscles of both limb girdles were atrophied. All deep reflexes were absent. The first electromyogram (E.M.G.) (November, 1976) pointed to motor-neurone loss with wide potentials exhibiting clear central driving, especially in deltoid muscles. Motor and sensory conduction velocities were normal. In February, 1977, the motor-neurone loss in deltoid muscle was extreme. Cerebrospinal fluid and plasma creatine kinase, alkaline phosphatase, and transaminases levels were normal. No signs of inflammatory or immunological disturbance were seen. The patient had been working for two years as a typesetter on a modern machine. Because of the remote possibility of lead intoxication the patient was tested for lead toxicity but nothing abnormal was found. To mobilise any stored lead in bones the patient was given penicillamine 300 mg three times a day for 5 days. Some slight improvement of muscle strength was noted by the fifth day. On March 6 continuous therapy with penicillamine was started (3 x 300 mg/day) and the patient is still on this regimen. After a month of this therapy the patient could walk on her heels or toes, raise her arms above her head, comb her hair, get up from a supine position without support, and lift a weight of about 9 kg. 2 months later she could squat and rise without help, carry a small child for an hour, walk for many hours, and do heavy agricultural work. In the E.M.G. (deltoid) besides the single-oscillation pattern of big potentials several polyphasic low-voltage potentials with
SIR,—We have
ment
5. Parkes, J. D., Bedard, P., Marsden, C. D. Lancet, 1976, ii, 155. 6. Cools, A. R., van Rossum, J. M. Psychopharmacologia, 1976, 45,
243.
