Paroxysmal Kinesigenic
Choreoathetosis and Brain-Stem
Atrophy To the Editor.\p=m-\Weread with interest the excellent article by Goodenough and coauthors (Archives 35:827-831, 1978). We recently saw a 22-year-old Lithuanian Jewish man who has classic paroxysmal kinesigenic choreoathetosis (PKC).
Computerized tomography scans showing severe enlargement of the fourth ventricle (top) and moderate enlargement of the circummesencephalic cisterns (bottom).
Report of a Case.\p=m-\This patient has had attacks since he was 14 years old. The attacks are more likely to occur if the patient is anxious. They can be suppressed by 5 to 10 s of voluntary ankle movement or by standing immobile for 5 to 10 s before walking. Results of physical and neurologic examinations were normal. After being distracted from rest with the request to quickly walk, he suffers grotesque dystonic posturing with facial grimacing and choreoathetoid movements of all limbs, lasting 15 to 20 s. He is perfectly lucid, although embarrassed, throughout. He is refractory to another attack for several minutes. The EEG was normal and the serum calcium level was 9.8 mg/dL. The compu¬ terized tomography (CT) scan sections showed severe enlargement of the fourth ventricle and moderate enlargement of the circummesencephalic cisterns (Figure). On CT sections not shown, the third and later¬ al ventricles were of small normal size and no cerebral or cerebellar cortical atrophie changes were present. The CT findings suggest brain-stem and possibly central cerebellar atrophy. The patient's 19-year-old sister has an idiopathic seizure disorder that started at age 6 months. She does not have choreoath¬ etosis. An EEG showed a 4-Hz spike-andwave
discharge during hyperventilation,
and a CT scan other siblings.
was
normal. There
are no
Comment—Previous cases of PKC have shown no convincing pathologic changes and pneumoencephalograms performed on a few have been reported as normal. Apparently no previously reported case has had a CT scan performed. The CT finding may be only coincidental, although if future scans on other patients show similar findings, they may help clarify the pathologic anatomy of this rare disorder. Robert T. Watson, MD William R. Scott, MD The Medical Center Clinic 8333 N Davis Hwy Pensacola, FL 32591
Carbamazepine-lnduced Oculogyric Crisis To the Editor.\p=m-\To our knowledge after a careful review of the English-
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/08/2015
language literature, including examination of literature by and communication with Geigy Pharmaceuticals (conversation with P. Gibney, MD, May 1979), oculogyric crisis due to administration of carbamazepine (Tegretol) has not been previously reported.1 Oculogyric crisis is a distressing symptom characterized by a forced upward gaze. It is, inter alia, an adverse effect of neuroleptic therapy.
Report of a Case.\p=m-\An8-year-old girl who suffered from grand mal seizures was admitted to a Detroit hospital in March 1977. The onset of seizures was 19 months earlier. The patient's mother had had a normal pregnancy, delivery, and postnatal course. Development had been normal. Results of neurological examination at the time of admission were normal, as was a computerized tomography scan. The patient was receiving phenytoin, 150 mg/day, and phenobarbital, 30 mg/day, on admission. Four EEGs made prior to the use of carbamazepine all showed sharp wave-and-spike activity, maximal midtemporal and posterior temporal. Carbamaze¬ pine was gradually added to the patient's regimen in the following manner: 100 mg daily on days 1 and 2; twice daily on days 3 through 6; three times daily on days 7 through 12; and four times daily on days 13 through 15. By day 15, the total daily dose was
400 mg.
day of administration of carbamazepine, an EEG showed that her condition was improved and showed only nonspecific changes. On the 13th day, the patient experienced three episodes of eyeblinking followed by severely painful eyerolling, dizziness, and headache. The first two episodes lasted 30 to 40 minutes, On the 12th
whereas the third lasted 20 minutes. Each episode consisted of an involuntary sus¬ tained conjugate movement of the eyes upward. A neurological examination at this time disclosed no other abnormal findings.
Diphenhydramine hydrochloride (Benadryl), 25 mg, was administered orally and
the symptoms subsided within 15 minutes, but returned on the following day. An EEG taken during these movements was unre¬ markable. The patient received 25 mg of
diphenhydramine hydrochloride orally again, with cessation of the symptom. On the 15th day of administration of carbama-
Choreoathetosis and Brain-Stem
Atrophy To the Editor.\p=m-\Weread with interest the excellent article by Goodenough and coauthors (Archives 35:827-831, 1978). We recently saw a 22-year-old Lithuanian Jewish man who has classic paroxysmal kinesigenic choreoathetosis (PKC).
Computerized tomography scans showing severe enlargement of the fourth ventricle (top) and moderate enlargement of the circummesencephalic cisterns (bottom).
Report of a Case.\p=m-\This patient has had attacks since he was 14 years old. The attacks are more likely to occur if the patient is anxious. They can be suppressed by 5 to 10 s of voluntary ankle movement or by standing immobile for 5 to 10 s before walking. Results of physical and neurologic examinations were normal. After being distracted from rest with the request to quickly walk, he suffers grotesque dystonic posturing with facial grimacing and choreoathetoid movements of all limbs, lasting 15 to 20 s. He is perfectly lucid, although embarrassed, throughout. He is refractory to another attack for several minutes. The EEG was normal and the serum calcium level was 9.8 mg/dL. The compu¬ terized tomography (CT) scan sections showed severe enlargement of the fourth ventricle and moderate enlargement of the circummesencephalic cisterns (Figure). On CT sections not shown, the third and later¬ al ventricles were of small normal size and no cerebral or cerebellar cortical atrophie changes were present. The CT findings suggest brain-stem and possibly central cerebellar atrophy. The patient's 19-year-old sister has an idiopathic seizure disorder that started at age 6 months. She does not have choreoath¬ etosis. An EEG showed a 4-Hz spike-andwave
discharge during hyperventilation,
and a CT scan other siblings.
was
normal. There
are no
Comment—Previous cases of PKC have shown no convincing pathologic changes and pneumoencephalograms performed on a few have been reported as normal. Apparently no previously reported case has had a CT scan performed. The CT finding may be only coincidental, although if future scans on other patients show similar findings, they may help clarify the pathologic anatomy of this rare disorder. Robert T. Watson, MD William R. Scott, MD The Medical Center Clinic 8333 N Davis Hwy Pensacola, FL 32591
Carbamazepine-lnduced Oculogyric Crisis To the Editor.\p=m-\To our knowledge after a careful review of the English-
Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 06/08/2015
language literature, including examination of literature by and communication with Geigy Pharmaceuticals (conversation with P. Gibney, MD, May 1979), oculogyric crisis due to administration of carbamazepine (Tegretol) has not been previously reported.1 Oculogyric crisis is a distressing symptom characterized by a forced upward gaze. It is, inter alia, an adverse effect of neuroleptic therapy.
Report of a Case.\p=m-\An8-year-old girl who suffered from grand mal seizures was admitted to a Detroit hospital in March 1977. The onset of seizures was 19 months earlier. The patient's mother had had a normal pregnancy, delivery, and postnatal course. Development had been normal. Results of neurological examination at the time of admission were normal, as was a computerized tomography scan. The patient was receiving phenytoin, 150 mg/day, and phenobarbital, 30 mg/day, on admission. Four EEGs made prior to the use of carbamazepine all showed sharp wave-and-spike activity, maximal midtemporal and posterior temporal. Carbamaze¬ pine was gradually added to the patient's regimen in the following manner: 100 mg daily on days 1 and 2; twice daily on days 3 through 6; three times daily on days 7 through 12; and four times daily on days 13 through 15. By day 15, the total daily dose was
400 mg.
day of administration of carbamazepine, an EEG showed that her condition was improved and showed only nonspecific changes. On the 13th day, the patient experienced three episodes of eyeblinking followed by severely painful eyerolling, dizziness, and headache. The first two episodes lasted 30 to 40 minutes, On the 12th
whereas the third lasted 20 minutes. Each episode consisted of an involuntary sus¬ tained conjugate movement of the eyes upward. A neurological examination at this time disclosed no other abnormal findings.
Diphenhydramine hydrochloride (Benadryl), 25 mg, was administered orally and
the symptoms subsided within 15 minutes, but returned on the following day. An EEG taken during these movements was unre¬ markable. The patient received 25 mg of
diphenhydramine hydrochloride orally again, with cessation of the symptom. On the 15th day of administration of carbama-
