✓ Persistent hemiballismus after stroke is often difficult to treat. The ballistic movement is sometimes so violent that progressive exhaustion results. The authors report two such cases, which were successfully treated by chronic thalamic stimulation. The lesions responsible for the ballistic movement in these patients were located near the subthalamic nucleus and in the putamen, respectively. The thalamic nucleus ventrolateralis and nucleus ventralis intermedius were stimulated with 0.2 to 0.3 msec pulses at 50 to 150 Hz and 4 to 7 V continuously during the day. Several weeks later, complete control of the hemiballismus was achieved during stimulation. The improvement was clearly not attributable to spontaneous recovery, because ballistic movement reappeared after termination of the stimulation. The stimulation has remained effective for more than 16 months in both cases without any serious complications. Chronic thalamic stimulation appears to be useful for controlling persistent hemiballismus, as it is for other involuntary movement disorders.
Report of two cases
Takashi Tsubokawa, Yoichi Katayama and Takamitsu Yamamoto
Toshinori Hasegawa, Yoshihisa Kida, Takenori Kato, Hiroshi Iizuka, Shunichiro Kuramitsu and Takashi Yamamoto
Little is known about long-term outcomes, including tumor control and adverse radiation effects, in patients harboring vestibular schwannomas (VSs) treated with stereotactic radiosurgery > 10 years previously. The aim of this study was to confirm whether Gamma Knife surgery (GKS) for VSs continues to be safe and effective > 10 years after treatment.
A total of 440 patients with VS (including neurofibromatosis Type 2) treated with GKS between May 1991 and December 2000 were evaluable. Of these, 347 patients (79%) underwent GKS as an initial treatment and 93 (21%) had undergone prior resection. Three hundred fifty-eight patients (81%) had a solid tumor and 82 (19%) had a cystic tumor. The median tumor volume was 2.8 cm3 and the median marginal dose was 12.8 Gy.
The median follow-up period was 12.5 years. The actuarial 5- and ≥ 10-year progression-free survival was 93% and 92%, respectively. No patient developed treatment failure > 10 years after treatment. According to multivariate analysis, significant factors related to worse progression-free survival included brainstem compression with a deviation of the fourth ventricle (p < 0.0001), marginal dose ≤ 13 Gy (p = 0.01), prior treatment (p = 0.02), and female sex (p = 0.02). Of 287 patients treated at a recent optimum dose of ≤ 13 Gy, 3 (1%) developed facial palsy, including 2 with transient palsy and 1 with persistent palsy after a second GKS, and 3 (1%) developed facial numbness, including 2 with transient and 1 with persistent facial numbness. The actuarial 10-year facial nerve preservation rate was 97% in the high marginal dose group (> 13 Gy) and 100% in the low marginal dose group (≤ 13 Gy). Ten patients (2.3%) developed delayed cyst formation. One patient alone developed malignant transformation, indicating an incidence of 0.3%.
In this study GKS was a safe and effective treatment for the majority of patients followed > 10 years after treatment. Special attention should be paid to cyst formation and malignant transformation as late adverse radiation effects, although they appeared to be rare. However, it is necessary to collect further long-term follow-up data before making conclusions about the long-term safety and efficacy of GKS, especially for young patients with VSs.
Wen-Zern Hwang, Takeshi Hasegawa, Haruhide Ito, Takashi Shimoji and Shinjiro Yamamoto
✓ A case of focal cerebral syphilitic gumma of the right temporal lobe is reported. Angiography showed moderate focal hypervascularity with stretched vessels, and irregularity of the vessel walls. Plain computerized tomography revealed an area of low density that enhanced strongly after intravenous administration of contrast medium.
Takashi Tsubokawa, Yoichi Katayama, Takamitsu Yamamoto, Teruyasu Hirayama and Seigou Koyama
✓ Analysis of the authors' experience over the last 10 years has indicated that excellent pain control has rarely been obtained by thalamic relay nucleus stimulation in patients with thalamic pain. In the present study, 11 patients with thalamic pain were treated by chronic stimulation of the precentral gyrus. In eight patients (73%), the stimulation system was internalized since excellent pain control was achieved during a 1-week test period of precentral gyrus stimulation. In contrast, no clear effect was noted or the original pain was even exacerbated by postcentral gyrus stimulation. The effect of precentral stimulation was unchanged in five patients (45%) for follow-up periods of more than 2 years. In the remaining three patients, the effect decreased gradually over several months. This outcome was significantly better than that obtained in an earlier series tested by the authors with thalamic relay nucleus stimulation (p < 0.05). The pain inhibition usually occurred at intensities below the threshold for production of muscle contraction (pulse duration 0.1 to 0.5 msec, intensity 3 to 8 V). When good pain inhibition was achieved, the patients reported a slight tingling or mild vibration sensation during stimulation projected in the same area of distribution as their pain.
The authors discuss the possibility that, in deafferentation pain, sensory neurons below the level of deafferentation cannot exert their normal inhibitory influences toward deafferented nociceptive neurons because of the development of aberrant connections. Thus, while stimulation of the first- to third-order sensory neurons at the level of the thalamic relay nucleus or below cannot bring about good pain inhibition in patients with thalamic pain, activation of hypothetical fourth-order sensory neurons through precentral stimulation may be able to inhibit deafferented nociceptive neurons within the cortex. None of the patients developed either observable or electroencephalographic seizure activity.
Yoichi Katayama, Takashi Tsubokawa, Tsuyoshi Maeda and Takamitsu Yamamoto
✓ In order to determine adequate therapeutic approaches for cavernous malformations of the third ventricle, the authors reviewed a series of five such malformations managed at their institution and nine others reported in the literature. Four subgroups were identified in terms of the site of origin and could be characterized by different clinical manifestations: visual field defects and endocrine function deficits in patients with malformations in the suprachiasmatic region (six cases); symptoms caused by hydrocephalus in those with malformations in the foramen of Monro region (five cases); and deficits of short-term memory in those with malformations in the lateral wall (two cases) or of the floor of the third ventricle (one case). Unlike cavernous malformations at other locations, malformations of the third ventricle frequently demonstrated rapid growth (43%) and mass effects (71%). The surgical or autopsy findings suggested that the growth was attributable to repeated intralesional hemorrhages. Extralesional hemorrhage was also not uncommon, occurring in 29% of patients. Such tendencies require the adoption of a more aggressive approach to this particular group of cavernous malformations as compared to those in other locations. The risks of regrowth and extralesional hemorrhage appear to be reduced only by complete excision. The surgical approaches adopted should be aimed at providing the best access to the site where the malformation has arisen. The translamina terminalis approach for cavernous malformations in the suprachiasmatic region, the transventricular or transcallosal interfornicial approaches for those in the foramen of Monro region and the transvelum interpositum approach for those in the lateral wall or the floor of the third ventricle appear to be appropriate. In order to select the adequate surgical approach, precise diagnosis of the site of origin is crucial. In addition to neuroimaging techniques, the patient's initial symptoms provide valuable information.
Toshinori Hasegawa, Yoshihisa Kida, Takenori Kato, Hiroshi Iizuka and Takashi Yamamoto
Gamma Knife surgery (GKS) has been a safe and effective treatment for small- to medium-sized vestibular schwannomas (VSs) over relatively long-term outcomes. However, even with recent radiosurgical techniques, hearing results following GKS remain unsatisfactory. The purpose of this study was to evaluate the hearing preservation rate as well as factors related to hearing preservation in patients with VSs and serviceable hearing who were treated with GKS.
Among patients with Gardner-Robertson (GR) Class I or II serviceable hearing and VSs treated with GKS between 1991 and 2009, 117 were evaluable via periodic MR imaging and audiometry.
The median age at the time of GKS was 52 years. Four patients (3%) had undergone prior surgery. Fifty-six patients (48%) had GR Class I hearing and 61 (52%) had GR Class II hearing at the time of GKS. The median tumor volume was 1.9 cm3. The median maximum and tumor margin radiation doses were 24 and 12 Gy, respectively. The median follow-up periods for MR imaging and audiometry were 74 and 38 months, respectively. The overall tumor control rate was 97.5%. Actuarial 3-, 5-, and 8-year hearing preservation rates were 55%, 43%, and 34%, respectively. On multivariate analysis, GR hearing class at the time of GKS and the mean cochlear dose affected hearing preservation significantly. In a limited number of patients who were treated using the most recent dose planning techniques and who had GR Class I hearing before treatment, the 3- and 5-year hearing preservation rates increased to 80% and 70%, respectively.
For the majority of patients with small- to medium-sized VSs, GKS was an effective and reasonable alternative to resection with satisfactory long-term tumor control. Factors related to hearing preservation included a GR Class I hearing pre-GKS and a lower mean cochlear radiation dose. To retain serviceable hearing, it is important to apply GKS treatment while patients retain GR Class I hearing.
Shuji Satoh, Nobutaka Yamamoto, Yoshinobu Kitagawa, Tsutomu Umemori, Takashi Sasaki and Takaaki Iida
✓ The authors report the case of a 59-year-old woman with progressive neck and arm pain that initially appeared in the neck and later extended to the shoulder and upper extremity. This pain was caused by compression of the cervical cord between the atlas and axis by the vertebral artery, and disappeared promptly following microvascular decompression.
Kazuaki Yamamoto, Hisashi Ito, Shigeru Fukutake, Takashi Odo, Tetsumasa Kamei, Toshio Yamaguchi and Takaomi Taira
Transcranial MR-guided focused ultrasound (MRgFUS) therapy is a less invasive form of stereotactic treatment for tremors and other movement disorders. Its stereotactic accuracy is ensured by stability of the stereotactic frame and MR table. The authors report a case wherein the patient’s movement was detected, and the MR images were repeated to continue the treatment.
A 72-year-old man with essential tremor underwent unilateral ventralis intermedius thalamotomy using MRgFUS. The stereotactic frame was correctly fixed to the patient’s skull and the table. During the seventh sonication, the patient pressed the emergency button and vomited several times. Before the eighth sonication, the patient’s movement was detected and was verified on coronal images. The MR images were repeated, and the treatment was successfully completed with significant improvement in the tremors. After treatment, it was discovered that the MR table was laterally unstable due to the absence of ball bearings, which should be present on both sides of the guide rail of the MR table. The ball bearings were attached to the reverse side of the table, and the table was stabilized. Stereotactic accuracy of MRgFUS is not only ensured by rigid fixation of the stereotactic frame, but also by stability of the MR table.
Tsuyoshi Sakurai, Hisao Seo, Naohito Yamamoto, Takashi Nagaya, Toshichi Nakane, Akio Kuwayama, Naoki Kageyama and Nobuo Matsui
✓ Clinically nonfunctioning pituitary adenomas have been thought to synthesize some pituitary hormones as shown by studies involving cell culture, immunocytochemistry, or measurement of hormone levels in tumor homogenates. Nevertheless, they are not associated with hypersecretion of pituitary hormones. To further clarify hormone synthesis in such pituitary adenomas, the presence of messenger ribonucleic acid (mRNA) of prolactin (PRL) growth hormone, and adrenocorticotropic hormone (ACTH) in the cytoplasm of 16 nonfunctioning adenomas was determined by means of a hybridization technique, and compared to the immunocytochemical findings. In three adenomas (19%) PRL mRNA was detected and in one case (6%) ACTH mRNA was detected. The hybridization technique appears to be more sensitive than immunohistochemistry for detection of specific mRNA's in assigning the hormone synthesis potential to clinically nonfunctioning tumors. The results suggest that PRL and ACTH are synthesized in some cases of clinically nonfunctioning pituitary adenomas and that hybridization techniques are useful to investigate hormone synthesis in pituitary adenomas. The ability to demonstrate PRL mRNA in tumor tissues allowed differentiation between hyperprolactinemia caused by synthesis of PRL in the tumor and that due to hypersecretion from the adjacent normal pituitary.