✓ Lesch—Nyhan syndrome (LNS) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. Patients with this syndrome are characterized by hyperuricemia, self-mutilation, developmental retardation, and movement disorders such as spasticity and dystonia. The authors performed bilateral chronic stimulation of the globus pallidus internus for control of dystonic movements in a 19-year-old man with LNS. His self-mutilating behavior unexpectedly disappeared after chronic stimulation. This is the first case of LNS that has been successfully treated with deep brain stimulation. The findings indicate that neurobehavioral features of this syndrome are either mediated in the basal ganglia pathways or secondary to the dystonia.
Takaomi Taira, Tomonori Kobayashi, and Tomokatsu Hori
Tomokatsu Hori, Hideo Terao, Tsuneyoshi Eguchi, and Masao Matsutani
✓ A case of a huge, rapidly enlarging, intraoral carotid aneurysm, presenting with dysphagia and dyspnea, was successfully treated by a combination of extracranial-intracranial bypass and internal carotid artery ligation. This case is unusual in that a tendency to bleed was observed for about 3 weeks after the operation. This is thought to be related to a consumption coagulopathy associated with the aneurysm.
Report of two cases
Hideo Terao, Tomokatsu Hori, Masao Matsutani, and Riki Okeda
✓ Two cases of cryptic vascular malformation that were not demonstrated by cerebral angiography were detected by computerized tomography. One of these patients had a cavernous angioma in the fourth ventricle with recurrent subarachnoid hemorrhages, and the other harbored a small arteriovenous malformation and intracerebral hematoma. The usefulness and limitations of computerized tomography in the identification of cryptic vascular malformations are discussed.
Kenichi Hirasawa, Hidetoshi Kasuya, and Tomokatsu Hori
Object. The importance of monitoring circulating blood volume (CBV) during perioperative management is widely recognized in critically ill patients. The purpose of this study was to investigate the change in CBV following craniotomy by using indocyanine-green pulse spectrophotometry.
Methods. Circulating blood volume and plasma hormones related to stress and fluid regulation were measured five times: preoperatively, immediately postoperatively, and 1, 2, and 7 days after craniotomy was performed in 17 patients with a brain tumor or an unruptured aneurysm.
The mean value of CBV preoperatively was 82 ml/kg, which decreased to 64 ml/kg (78%) immediately postoperatively and gradually recovered to 82 ml/kg on Day 7 postsurgery (p = 0.0069). The mean values of adrenaline, noradrenaline, arginine vasopressin, renin, and aldosterone were highest immediately postoperatively. The mean intraoperative balances of water and sodium were 1090 ml and 113 mEq, respectively. Partial correlation coefficients of CBV to noradrenaline and serum sodium during the entire study were −0.430 (p = 0.0036) and 0.418 (p = 0.0048), respectively.
Conclusions. Attention should be paid to decreased CBV following craniotomy, which is caused by the shift of fluid to interstitial spaces due to surgical stress. Hypovolemia can be suspected from a postoperative decrease in serum sodium.
Michiharu Tanabe, Takashi Watanabe, and Tomokatsu Hori
✓ The case of a 39-year-old man with von Recklinghausen's disease, presenting with emaciation and a marked increase in serum growth hormone concentration, is presented. Neuroradiological and histological examination confirmed anaplastic astrocytoma in the optic chiasm-hypothalamic region. This is a rare case of diencephalic syndrome and von Recklinghausen's occurring together in an adult.
Takaomi Taira, Tomonori Kobayashi, Kenji Takahashi, and Tomokatsu Hori
Object. The Bertrand selective peripheral denervation for cervical dystonia (CD) has been well described, and its effectiveness and safety are established. It is, however, always accompanied by postoperative sensory loss in the C-2 region. Intraoperative bleeding from epidural venous plexuses may also be problematic. The authors developed a new denervation procedure with which to avoid such complications and compared the surgery-related results with those of the traditional Bertrand operation.
Methods. The new procedure consists of intradural rhizotomy of the anterior C-1 and C-2 nerve roots, extradural peripheral ramisectomy from C-3 to C-6, and selective section of peripheral branches of the accessory nerve to the sternocleidomastoid muscle. This procedure was performed in 30 patients (Group A). The results of this procedure were compared with those obtained in a matched group of 31 patients in the authors' series who underwent Bertrand denervation (Group B).
Changes of CD rating score at 6-month follow up did not differ between the two groups. In one patient in Group A a C-2 sensory deficit was found, whereas C-2 sensory deficits were demonstrated in all the patients in Group B. No patients in Group A and four patients in Group B experienced occipital neuralgia. The operative time was significantly shorter in Group A. The mean intraoperative blood loss was 115 ± 30 ml (± standard deviation) in Group A and 233 ± 65 ml in Group B (p < 0.005).
Conclusions. Although symptomatic improvement is the same after the Bertrand operation, the authors' new procedure for CD was associated with a lower incidence of complications and significant decrease of intraoperative blood loss.
Tomokatsu Hori, Takanori Fukushima, Hideo Terao, Kintomo Takakura, and Keiji Sano
✓ The authors have developed a fluoroscopy-assisted technique of percutaneous radiofrequency facial nerve coagulation at or near the stylomastoid foramen for management of facial spasm. The details of the procedure and the operative results in the initial series of 27 cases of facial spasm are described. The series included six men and 21 women, aged from 16 to 73 years. Twenty-four patients had classical, intractable, persistent hemifacial spasm, one had an unusual bilateral facial spasm, and one a postparalytic facial spasm. The age of onset varied from 16 years to 70 years. The duration of the symptoms ranged from 3 months to 11 years. Ten patients had undergone previous blunt-needle compression of the facial nerve from one to 10 times. The operative results in the 27 patients have so far been excellent. The longest follow-up has been 1½ years. Although partial facial weakness was present in 60% of the cases, it invariably disappeared within 1 to 4 months. Only three patients experienced recurrence of spasm. In two of them, recurrence followed lowtemperature coagulation. The procedure is simple, easy to perform, and extremely effective. It can be performed under local anesthesia in the outpatient clinic.
Satoshi Tanaka, Tadashi Nagashima, Shinya Manaka, Tomokatsu Hori, and Shigeru Yasumoto
✓ The effect of recombinant human interleukin-1 (rHuIL-1) derivatives on human glioma cell lines was examined in vitro. Five glioma cell lines, U-251 MG, U-373 MG, U-87 MG, A-172, and T98G, were incubated in medium containing 1% fetal calf serum and various concentrations of different types of rHuIL-1: OCT-43 (rHuIL-1β), OCT-7000 (rHuIL-1α), and OCT-8000 (rHuIL-1α). The high-affinity IL-1 receptors were expressed in the U-251 MG and U-373 MG cell lines, and rHuIL-1 was found to suppress cell growth and to induce morphological differentiation of these cell lines. Growth inhibition occurred in a dose-dependent manner in concentrations or rHuIL-1 ranging between 1 and 100 ng/ml. Interestingly, rHuIL-1 induced a transient growth of glioma cells shortly after administration, then suppressed cell growth with accompanying elongation of cytoplasmic processes. This unique process of transient growth stimulation followed by growth suppression was parallel to the efficacy of bromodeoxyuridine uptake in the rHuIL-1-treated cells. Concomitantly, accumulation of glial fibrillary acidic protein and cyclic adenosine monophosphate contents was observed in four glioma cell lines. Continuous rHuIL-1 treatment for longer than 30 days elicited irreversible astrocytic terminal differentiation. These results indicate that IL-1 is an effector on the growth regulation of glioma cells, resulting in astrocytic differentiation in vitro.
Kotaro Nakaya, Motohiro Hayashi, Masahiro Izawa, Taku Ochiai, Tomokatsu Hori, and Kintomo Takakura
Stereotactic radiosurgery for brain metastasis has become one of the standard treatment options in recent years. Some patients must undergo repeated stereotactic radiosurgery for new lesions. The authors retrospectively reviewed their data to estimate how soon the patients undergo repeated radiosurgery for new lesions.
Between October 1999 and March 2006, 1081 patients with brain metastases underwent Gamma Knife surgery (GKS) at Tokyo Women's Medical University. One hundred and forty-nine patients in whom GKS had been performed two or more times were evaluated. There were 68 men and 81 women with a median age of 61 years (range 29–90 years). The authors analyzed data on patient age, number of treated lesions, and period between GKSs. Follow-up imaging was performed in almost all patients every 2 to 3 months after GKS.
The number of lesions treated in a single session varied from one to 35. The median interval between GKSs was 26 weeks (range 3–175 weeks) for patients with breast cancer and 23 weeks (range 4–179 weeks) for patients with non–small cell lung carcinoma.
It would appear that follow-up imaging studies should be obtained every 2 to 3 months after GKS to monitor patients for tumor recurrence.