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The EEG in a case of periodic hypersomnia
Electroencephalography and Clinical Neurophysiology Elsevier Publishing Company, Amsterdam - Printed in The Netherlands
605
THE LEG IN A CASE OF PERIODIC HYPERSOMNIA V. R . THACORE, MOHAMMAD AHMED AND ~[AN OSWALD
Department of Medicine, King George'sMedical College, University of Lucknow, Lucknow (India) and Department of Psychiatry, University of Edinburgh, Edinburgh (Great Britain) (Accepted for publication: April 8, 1969)
A number of cases of apparent abnormal sleepiness with overeating were described by Kleine (1925) and Levin (1929), after which other reports appeared describing patients with clinical features of greater or less similarity, mainly male adolescents. In a critical review, Critchley (1962) considered fifteen of the cases to conform to his criteria of a syndrome, and detailed nine new cases. He rejected many reports, including all female cases, though Duffy and Davison (1968) have described a female case of the "Kleine-Levin syndrome" which otherwise conformed to Critchley's criteria. Not all Critchley's own cases showed overeating. Their only remaining comm o n feature was "sleep", described as excessive during self-limiting periods. The present case is reported because of its similarity to many of the cases in the literature, because of the early onset age and because the LEG was recorded in a n attack and was not characteristic of sleep.
meals (during each he would often fall asleep). He did not overeat but continually chewed his lips. There was no incontinence. He was disorientated and indifferent to his surroundings, his memory appeared defective and he just stared when asked questions. He said he felt different but denied hallucinations or delusions. He repeatedly dozed off during interviews and appeared to sleep a total of 20 hours per day. The L E G (Fig. 1) recorded during the "sleep" state was dominated by generalized and symmetrical theta and delta activity, including bursts of delta waves of up to 220/~V at 2.5-3 c/sec, lasting about 10 sec. Brief periods of alpha rhythm were interspersed with theta activity. Slow wave activity continued when the eyes were open. No sleep spindles were present. Six months later his father reported that he had remained well and resumed school. Contact was then lost.
CASE
DISCUSSION
A boy, aged 13, had had bouts of excessive "sleep" for 5 years. The first episode lasted 7 days, as did a second 3 months later, and after each he recovered spontaneously. Two years later they began again, lasting 10-15 days and recurring about monthly, always with subsequent amnesia. He was alert and sociable in the intervals. The episode during which the patient was brought to the hospital was the second in one month. The family history revealed little of relevance apart from an uncle who had attempted suicide after asocial acts. There was no history of significant physical disorder in the patient who was, however, described as stubborn and short-tempered. He was of average build, afebrile, pulse 80/min, blood pressure 120/70 mm Hg and drowsy when first seen. Haemoglobin 12 g ~ , blood picture normal, with leucocytes 12,000/ram a, neutrophils 66 ~ , lymphocytes 32 ~ . Fasting blood sugar 48 mg ~ . Liver function tests, serum cholesterol and protein, normal. Corrected ESR (Wintrobe) 6 mm for first hour, packed cell volume 38 ~ . Cerebro-spinal fluid clear, normal tension, proteins 30 m g ~ , chloride 650 m g ~ , sugar 70 m g ~ . cells 5/mm a. Wassermann reaction and Venereal Disease Reference Laboratory serological test negative. X-ray chest and skull, normal. He was difficult to rouse and would follow instructions for only a few seconds before lapsing back into his inattentive state. He lay curled up, was irritable if disturbed, but would waken or be roused for toilet and
Prolonged sleep-like states may be found in association with lesions of the brain-stem, in metabolic disorders and psychiatric conditions such as hysteria and depressive or catatonic stupor. In the present patient the low single fasting blood sugar suggests the possibility of a hypoglycaemic state but, with no clinical signs of hypoglycaemia and intake of food being without effect, hypoglycaemia as a primary aetiological factor appears out of the question. The youth looked as if he were asleep but, in contrast to true sleep, he could not be fully roused and his L E G was not that of sleep. Only two of Critchley's (1962) cases had LEGs performed during an attack and in neither case was the LEG that of sleep or, apparently, even that of drowsiness. His Case 7 had alpha rhythm, a hallmark of wakefulness, mixed with "post-central moderate voltage, slow waves", some of the last still being present after the attack. Case 3 "while he was in a state of somnosis" had alpha rhythm and W.A. Cobb was quoted as being "surprised at the slightness of the change during an attack compared with those of a comparable state of natural drowsiness". Critchley nevertheless wrote "the sleep does not differ in any obvious fashion from the normal". Criticism of Critchley's criteria of sleep has been made elsewhere (Oswald 1969). Bonkalo (1968) described what he considered a case of the "Kleine-Levin syndrome" showing "true sleep". However, the description of "episodic or continuous high
Electroenceph. clin. Neurophysiol., 1969, 27:605-606
606
v.R.
THACORE et al.
~t
luc
Fig. 1 A representative excerpt of the EEG of the patient during the "sleep" state. Bursts of diffuse delta waves of high voltage are interspersed in a record consisting predominantly of theta rhythm. No sleep spindles are present. Calibrations: 50 ,u¥ and 1 sec. amplitude 5-6 cycles per second slow waves occurring in both frontal and temporal leads of the LEG" is not that of sleep. The only instance of a sleep EEG, with typical spindles illustrated, is to be found in a case of the Kleine-Levin syndrome, unmentioned by Critchley (1962), and described by Rosenk6tter and Wende 0955). The literature contains reports of patients of both sexes, not all of whom were adolescents, who suffered recurrent periods of altered consciousness, with inertia and unresponsiveness, appearing and disappearing spontaneously. Only some exhibited overeating and among those who looked as if they were asleep, a number would seem to have been awake. It would appear that any attempt to delineate a nosological entity must, in large measure, be dictated by idiosyncratic preference. SUMMARY A case of periodic hypersomnia, conforming to descriptions of the Kleine-Levin syndrome, is reported in a male of 13, the age of onset being 8 years. Overeating did not occur. The attacks, which lasted 1-2 weeks and ended spontaneously, were accompanied by a state which resembled sleep, but from which arousal to normal alertness was not possible. In an attack the EEG showed diffuse delta and theta abnormalities which did not resemble the EEG of sleep. Attention is drawn to the heterogeneity of other published cases. RI~SUME L'EEG DANS UN CAS D'HYPERSOMNIE PERIODIQUE Les auteurs rapportent un cas correspondant aux descriptions de syndrome de Kleine-Levin chez un sujet
nffde de 13 ans, syndrome ayant debut6 ~t l'/tge de 8 ans. L'hyperphagie n'a pas 6t6 notde. Les crises qui ont persiste pendant 1-2 semaines et se sont termin6es spontan6ment, s'accompagnaient d'un 6tat ressemblant au sommeil; mais l'6veil 5. un niveau vigile normal n'6tait pas possible, et au cours de Fun de ces 6tats, I'EEG montrait des anomalies theta et delta diffuses qui ne ressemblaient pas b. I'EEG du sommeil. L'het6rog6n6it6 des autres cas publi6s est soulign6e. We thank Dr. B. C. Chatterjee for his co-operation. REFERENCES BONKALO, A. Hypersomnia. Brit. J. Psychiat., 1968, 114: 69-75. CRITCHLEY, M. Periodic hypersomnia and megaphagia in adolescent males. Brain, 1962, 85: 627-656. DUFFY, J. P. and DAV1SON, K. A female case of the Kleine-Levin syndrome. Brit. J. Psychiat., 1968, 114: 77-84. KLE1NE, W. Periodische Schlafsucht. Mschr. Psychiat. Neurol., 1925, 57: 285-320. LEVIN, M. Narcolepsy and other varieties of morbid somnolence. Arch. Neural. Psychiat. (Chic.), 1929, 22: 1172-1200. OSWALD, 1. Sleep and its disorders. In P. J. VINKEN and G. W. BRUYN (Eds.), Handbook of clinical neurology, Vol. 3. North-Holland Publ. Co., Amsterdam, 1969: 80-111. ROSENKOTTER, L. und WENDE, S. EEG-Befunde beim Kleine-Levin-Syndrom. l~Ischr. Psychiat. Neurol., 1955, 130: 107-122.
Reference: THACORE,V. R.., AHMED, M. and OSWALD,I. The LEG in a case of periodic hypersomnia. Electroenceph. clin. Neurophysiol., 1969, 27: 605-606.
605
THE LEG IN A CASE OF PERIODIC HYPERSOMNIA V. R . THACORE, MOHAMMAD AHMED AND ~[AN OSWALD
Department of Medicine, King George'sMedical College, University of Lucknow, Lucknow (India) and Department of Psychiatry, University of Edinburgh, Edinburgh (Great Britain) (Accepted for publication: April 8, 1969)
A number of cases of apparent abnormal sleepiness with overeating were described by Kleine (1925) and Levin (1929), after which other reports appeared describing patients with clinical features of greater or less similarity, mainly male adolescents. In a critical review, Critchley (1962) considered fifteen of the cases to conform to his criteria of a syndrome, and detailed nine new cases. He rejected many reports, including all female cases, though Duffy and Davison (1968) have described a female case of the "Kleine-Levin syndrome" which otherwise conformed to Critchley's criteria. Not all Critchley's own cases showed overeating. Their only remaining comm o n feature was "sleep", described as excessive during self-limiting periods. The present case is reported because of its similarity to many of the cases in the literature, because of the early onset age and because the LEG was recorded in a n attack and was not characteristic of sleep.
meals (during each he would often fall asleep). He did not overeat but continually chewed his lips. There was no incontinence. He was disorientated and indifferent to his surroundings, his memory appeared defective and he just stared when asked questions. He said he felt different but denied hallucinations or delusions. He repeatedly dozed off during interviews and appeared to sleep a total of 20 hours per day. The L E G (Fig. 1) recorded during the "sleep" state was dominated by generalized and symmetrical theta and delta activity, including bursts of delta waves of up to 220/~V at 2.5-3 c/sec, lasting about 10 sec. Brief periods of alpha rhythm were interspersed with theta activity. Slow wave activity continued when the eyes were open. No sleep spindles were present. Six months later his father reported that he had remained well and resumed school. Contact was then lost.
CASE
DISCUSSION
A boy, aged 13, had had bouts of excessive "sleep" for 5 years. The first episode lasted 7 days, as did a second 3 months later, and after each he recovered spontaneously. Two years later they began again, lasting 10-15 days and recurring about monthly, always with subsequent amnesia. He was alert and sociable in the intervals. The episode during which the patient was brought to the hospital was the second in one month. The family history revealed little of relevance apart from an uncle who had attempted suicide after asocial acts. There was no history of significant physical disorder in the patient who was, however, described as stubborn and short-tempered. He was of average build, afebrile, pulse 80/min, blood pressure 120/70 mm Hg and drowsy when first seen. Haemoglobin 12 g ~ , blood picture normal, with leucocytes 12,000/ram a, neutrophils 66 ~ , lymphocytes 32 ~ . Fasting blood sugar 48 mg ~ . Liver function tests, serum cholesterol and protein, normal. Corrected ESR (Wintrobe) 6 mm for first hour, packed cell volume 38 ~ . Cerebro-spinal fluid clear, normal tension, proteins 30 m g ~ , chloride 650 m g ~ , sugar 70 m g ~ . cells 5/mm a. Wassermann reaction and Venereal Disease Reference Laboratory serological test negative. X-ray chest and skull, normal. He was difficult to rouse and would follow instructions for only a few seconds before lapsing back into his inattentive state. He lay curled up, was irritable if disturbed, but would waken or be roused for toilet and
Prolonged sleep-like states may be found in association with lesions of the brain-stem, in metabolic disorders and psychiatric conditions such as hysteria and depressive or catatonic stupor. In the present patient the low single fasting blood sugar suggests the possibility of a hypoglycaemic state but, with no clinical signs of hypoglycaemia and intake of food being without effect, hypoglycaemia as a primary aetiological factor appears out of the question. The youth looked as if he were asleep but, in contrast to true sleep, he could not be fully roused and his L E G was not that of sleep. Only two of Critchley's (1962) cases had LEGs performed during an attack and in neither case was the LEG that of sleep or, apparently, even that of drowsiness. His Case 7 had alpha rhythm, a hallmark of wakefulness, mixed with "post-central moderate voltage, slow waves", some of the last still being present after the attack. Case 3 "while he was in a state of somnosis" had alpha rhythm and W.A. Cobb was quoted as being "surprised at the slightness of the change during an attack compared with those of a comparable state of natural drowsiness". Critchley nevertheless wrote "the sleep does not differ in any obvious fashion from the normal". Criticism of Critchley's criteria of sleep has been made elsewhere (Oswald 1969). Bonkalo (1968) described what he considered a case of the "Kleine-Levin syndrome" showing "true sleep". However, the description of "episodic or continuous high
Electroenceph. clin. Neurophysiol., 1969, 27:605-606
606
v.R.
THACORE et al.
~t
luc
Fig. 1 A representative excerpt of the EEG of the patient during the "sleep" state. Bursts of diffuse delta waves of high voltage are interspersed in a record consisting predominantly of theta rhythm. No sleep spindles are present. Calibrations: 50 ,u¥ and 1 sec. amplitude 5-6 cycles per second slow waves occurring in both frontal and temporal leads of the LEG" is not that of sleep. The only instance of a sleep EEG, with typical spindles illustrated, is to be found in a case of the Kleine-Levin syndrome, unmentioned by Critchley (1962), and described by Rosenk6tter and Wende 0955). The literature contains reports of patients of both sexes, not all of whom were adolescents, who suffered recurrent periods of altered consciousness, with inertia and unresponsiveness, appearing and disappearing spontaneously. Only some exhibited overeating and among those who looked as if they were asleep, a number would seem to have been awake. It would appear that any attempt to delineate a nosological entity must, in large measure, be dictated by idiosyncratic preference. SUMMARY A case of periodic hypersomnia, conforming to descriptions of the Kleine-Levin syndrome, is reported in a male of 13, the age of onset being 8 years. Overeating did not occur. The attacks, which lasted 1-2 weeks and ended spontaneously, were accompanied by a state which resembled sleep, but from which arousal to normal alertness was not possible. In an attack the EEG showed diffuse delta and theta abnormalities which did not resemble the EEG of sleep. Attention is drawn to the heterogeneity of other published cases. RI~SUME L'EEG DANS UN CAS D'HYPERSOMNIE PERIODIQUE Les auteurs rapportent un cas correspondant aux descriptions de syndrome de Kleine-Levin chez un sujet
nffde de 13 ans, syndrome ayant debut6 ~t l'/tge de 8 ans. L'hyperphagie n'a pas 6t6 notde. Les crises qui ont persiste pendant 1-2 semaines et se sont termin6es spontan6ment, s'accompagnaient d'un 6tat ressemblant au sommeil; mais l'6veil 5. un niveau vigile normal n'6tait pas possible, et au cours de Fun de ces 6tats, I'EEG montrait des anomalies theta et delta diffuses qui ne ressemblaient pas b. I'EEG du sommeil. L'het6rog6n6it6 des autres cas publi6s est soulign6e. We thank Dr. B. C. Chatterjee for his co-operation. REFERENCES BONKALO, A. Hypersomnia. Brit. J. Psychiat., 1968, 114: 69-75. CRITCHLEY, M. Periodic hypersomnia and megaphagia in adolescent males. Brain, 1962, 85: 627-656. DUFFY, J. P. and DAV1SON, K. A female case of the Kleine-Levin syndrome. Brit. J. Psychiat., 1968, 114: 77-84. KLE1NE, W. Periodische Schlafsucht. Mschr. Psychiat. Neurol., 1925, 57: 285-320. LEVIN, M. Narcolepsy and other varieties of morbid somnolence. Arch. Neural. Psychiat. (Chic.), 1929, 22: 1172-1200. OSWALD, 1. Sleep and its disorders. In P. J. VINKEN and G. W. BRUYN (Eds.), Handbook of clinical neurology, Vol. 3. North-Holland Publ. Co., Amsterdam, 1969: 80-111. ROSENKOTTER, L. und WENDE, S. EEG-Befunde beim Kleine-Levin-Syndrom. l~Ischr. Psychiat. Neurol., 1955, 130: 107-122.
Reference: THACORE,V. R.., AHMED, M. and OSWALD,I. The LEG in a case of periodic hypersomnia. Electroenceph. clin. Neurophysiol., 1969, 27: 605-606.