| TI: [Circadian rhythm disturbance after radiotherapy for brain tumor in infantile period--clinical effect of L-thyroxine and vitamin B12] |
| AU: Kubota-M; Shinozaki-M; Sasaki-H |
| SO: No-To-Shinkei. 1993 Aug; 45(8): 759-63 |
| PY: 1993 |
| AB: We reported here 19-year-old man suffering from circadian sleep-wake (S-W) rhythm disturbance after total tumor resection and whole brain irradiation. This 19-year-old man was diagnosed as having astrocytoma in the right temporal lobe by CT scan and angiography at the age of 6 months. After total tumor resection and whole brain irradiation (60Co 60 Gy), he showed profound psychomotor retardation, endocrinologic dysfunction including hypothyroidism and growth hormone deficiency, and sleep-wake rhythm disturbance. At the age of 19, brain MRI revealed asymmetrical low intensity in the hypothalamic region. On endocrinological examination panhypopituitarism due to primary hypothalamic lesion was evident. His S-W rhythm was disturbed showing a dispersed type sleep, i.e., sleep periods were dispersedly distributed throughout the 24 hours. So he showed a lethargic tendency in the daytime. All-day polysomnography revealed abnormal sleep structure such as the absence of sleep spindle and hump, peripheral apnea, snoring and low oxygen saturation. After L-thyroxine supplementation his daily activity improved gradually. The decrease in short time sleep and tendency of a free-running rhythm were observed and oxygen saturation improved remarkably. Peripheral apnea and snoring disappeared. The wakening effect of L-thyroxine administration may be due to improvement of hypothyroidism symptom such as myxoedematous pharynx. In addition, it seems related to the alteration of the central S-W rhythm regulation, because free-running rhythm appeared after L-thyroxine administration. Vitamin B12 (VB12), which has been reported to be effective for sleep-wake rhythm disorders, was not effective for our patient's free-running rhythm.(ABSTRACT TRUNCATED AT 250 WORDS) |
| TI: [Lennox syndrome associated with severe cerebellar dysfunction and peripheral neuropathy] |
| Kubota-M; Nagata-J |
| No-To-Shinkei. 1993 Jul; 45(7): 669-71 |
| PY: 1993 |
| AB: We reported a 33-year-old man with Lennox syndrome of 26 years' duration associated with unusual symptom complexes such as severe cerebellar ataxia and dysarthria, and peripheral neuropathy. His convulsive disorder was very intractable despite multiple anticonvulsants including phenytoin (PHT), phenobarbital (PB), primidone (PRM), valproate and so on. At the age of 25 he was no longer able to walk without help. PHT blood levels were kept almost within the therapeutic range, while PB blood levels tended to be greater than the therapeutic range. Needle EMG study revealed denervation pattern. Motor conduction velocity of the peroneal nerve was 25.2 m/s and sensory conduction velocity of the sural nerve could not be elicited. Brain CT and MRI showed the marked cerebellar atrophy predominant in the vermis. To our knowledge there were no previously reported cases of Lennox syndrome associated with such cerebellar dysfunctions and peripheral neuropathy. From the clinical course and laboratory findings, metabolic disorders and degenerative diseases were ruled out. We consider his cerebellar symptoms and peripheral neuropathy could be attributable to the long-term use of multiple anticonvulsants, i.e. PHT in combination with PB and PRM. These symptoms seem to be irreversible, because our patient's condition did not change after PHT and PB dose reduction, and discontinuation of PRM. |
Edited by Toshio Hishi