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Scientific meeting of the Netherlands Society of Child Neurology held in Heeze, 7th of April 1989
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21 patients with Spina Bifida Occulta (S.B.O.) - 22 patients with Spina Bifida Aperta @B.A.) In the S.B .O. group 4 patients were operated at a young age in order to prevent the development of neurological symptoms. All the other patients were operated symptomatically. Problems of motor dysfunction were somewhat more pronounced in the S.B.A. group, apart from that the preoperative signs and symptoms were comparable. Imaging of the tethered cord syndrome was performed in most patients by C.T. myelography and in a few by Magnetic Resonance imaging. The post-operative complication rate was low and of minor importance. It was the purpose of the operation to stop a further neurological deterioration. When we compare the post-operative results
The Kleine-Levin
in both groups we found: (follow-up at least 6 months). - In the S.B.O. group (21 patients) 7 patients improved, 6 patients stabilised and 4 patients showed a deterioration which improved after reoperation in 2 patients. - In the S.B.A. group (22 patients)
14 patients improved, 4 patients stabilized and in 3 patients a post-operative deterioration occurred. Of the last group 2 patients were recently re-operated.
The improvement occurred within half a year after operation in both groups. In our opinion operation for a symptomatic tethered cord syndrome is indicated whether the cause of the tethering is a post-meningomyelocele repair scar or not. Preventive operation of patients with spina bifida occulta remains mandated.
syndrome
F. Visscher, L.M.E. Boer, Ch. Njiokiktjien den)
Smit, F. Smith, F. (Amsterdam, Lei-
The combination of periodic somnolence and excessive eating, often coupled with a disturbance of sexual impulse control, was described by Kleine in 1925 and by Levin in 1936. Mostly boys are affected, usually at the age of 10 to 20 years. The cause of the syndrome is unknown, in some cases a relationship between infectious disease or traumatic brain damage has been presumed. A dysfunction of the hypothalamus and associated structures is suspected.
A peculiar feature is the relapsing character: symptomatic periods are followed by symptomfree intervals. The syndrome has a rather favourable prognosis. The symptoms can be relieved by amphetamines, methylphenidate and probably lithiumcarbonate. We present two boys, aged 12 and 13 years, who developed the Kleine-Levin syndrome after an influenza-like febrile illness. After a three years’ treatment with methylphenidate, they are still suffering, but the symptoms could be partially relieved. The symptom-free intervals increased from one week to several months.
Right-left differences in neonatal cerebral blood flow
R.A. Mullaart, J.C.W. Hopman,A.F.J. de Haan, J.J. Rotteveel, 0. Daniels (Nijmeget-0 Neonatal cerebrovascular accidents occur preferentially in the left cerebral hemisphere. Therefore we investigated cerebral blood flow right-left differences by means of transfontanel-
lar echo-Doppler measurement of blood velocity in the supraclinoid segment of both internal carotid arteries. Thirty seven preterm neonates were examined repeatedly: within 12 hours after birth, between 12 & 24 hours and on the 2nd, 3rd and 5th day of extrauterine life. We found systematic lower velocities at the left side, with differences of: 1,3 cm s’ for systol365
21 patients with Spina Bifida Occulta (S.B.O.) - 22 patients with Spina Bifida Aperta @B.A.) In the S.B .O. group 4 patients were operated at a young age in order to prevent the development of neurological symptoms. All the other patients were operated symptomatically. Problems of motor dysfunction were somewhat more pronounced in the S.B.A. group, apart from that the preoperative signs and symptoms were comparable. Imaging of the tethered cord syndrome was performed in most patients by C.T. myelography and in a few by Magnetic Resonance imaging. The post-operative complication rate was low and of minor importance. It was the purpose of the operation to stop a further neurological deterioration. When we compare the post-operative results
The Kleine-Levin
in both groups we found: (follow-up at least 6 months). - In the S.B.O. group (21 patients) 7 patients improved, 6 patients stabilised and 4 patients showed a deterioration which improved after reoperation in 2 patients. - In the S.B.A. group (22 patients)
14 patients improved, 4 patients stabilized and in 3 patients a post-operative deterioration occurred. Of the last group 2 patients were recently re-operated.
The improvement occurred within half a year after operation in both groups. In our opinion operation for a symptomatic tethered cord syndrome is indicated whether the cause of the tethering is a post-meningomyelocele repair scar or not. Preventive operation of patients with spina bifida occulta remains mandated.
syndrome
F. Visscher, L.M.E. Boer, Ch. Njiokiktjien den)
Smit, F. Smith, F. (Amsterdam, Lei-
The combination of periodic somnolence and excessive eating, often coupled with a disturbance of sexual impulse control, was described by Kleine in 1925 and by Levin in 1936. Mostly boys are affected, usually at the age of 10 to 20 years. The cause of the syndrome is unknown, in some cases a relationship between infectious disease or traumatic brain damage has been presumed. A dysfunction of the hypothalamus and associated structures is suspected.
A peculiar feature is the relapsing character: symptomatic periods are followed by symptomfree intervals. The syndrome has a rather favourable prognosis. The symptoms can be relieved by amphetamines, methylphenidate and probably lithiumcarbonate. We present two boys, aged 12 and 13 years, who developed the Kleine-Levin syndrome after an influenza-like febrile illness. After a three years’ treatment with methylphenidate, they are still suffering, but the symptoms could be partially relieved. The symptom-free intervals increased from one week to several months.
Right-left differences in neonatal cerebral blood flow
R.A. Mullaart, J.C.W. Hopman,A.F.J. de Haan, J.J. Rotteveel, 0. Daniels (Nijmeget-0 Neonatal cerebrovascular accidents occur preferentially in the left cerebral hemisphere. Therefore we investigated cerebral blood flow right-left differences by means of transfontanel-
lar echo-Doppler measurement of blood velocity in the supraclinoid segment of both internal carotid arteries. Thirty seven preterm neonates were examined repeatedly: within 12 hours after birth, between 12 & 24 hours and on the 2nd, 3rd and 5th day of extrauterine life. We found systematic lower velocities at the left side, with differences of: 1,3 cm s’ for systol365