[Stereotactic Radiosurgery for Trigeminal Neuralgia].

Stereotactic radiosurgery(SRS)performed with Gamma Knife or CyberKnife has been reported to be effective in treating trigeminal neuralgia(TN). Microvascular decompression is the first choice of treatment for patients with trigeminal neuralgia who are difficult to treat with drugs because of its high efficacy, with a pain relief rate of 70%-80% after 5 years. The pain relief rate of TN treated with SRS is approximately 50%-60% after 5 years, which is less than that of MVD. SRS is also inferior to surgery, causing more frequent sensory disturbances in the trigeminal nerve area(6%-20%). However, the serious complications, severe morbidity and mortality, associated with SRS are quite rare. SRS is an extremely minimally invasive treatment that does not require general anesthesia and can be used to treat TN with short-term hospitalization or outpatient visits.

[Image Diagnosis for Hemifacial Spasm].

Cisternography using heavy T2-weighted images from 3-Tesla magnetic resonance imaging(MRI)and three-dimensional time-of-flight MR angiography(3D TOF MRA)is useful for identifying conflicting vessels in primary hemifacial spasm(HFS). Cisternography provides high-signal images of the cerebrospinal fluid and low-signal images of the cranial nerves and cerebral blood vessels, whereas 3D TOF MRA provides high-signal images with only vascular information. The combination of these two methods increases the identification rate of conflicting vessels. The neurovascular conflict(NVC)site in HFS is where the facial nerve exits the brainstem. However, on MRI, the true NVC site is often more proximal than the facial nerve attachment to the brainstem. On preoperative MRI, it is important to not miss the blood vessels surrounding the proximal portion of the facial nerve. If multiple compression vessels or deep vessels are located in the supraolivary fossette, they may be missed. Coronal section imaging and multiplanar reconstruction(MPR)minimize the chances of missing a compression vessel. Preoperative MRI and CT can also provide various other information, such as volume of the cerebellum, presence of emissary veins, shape of the petrosal bone, and size of the flocculus.

Far-field potential of the compound muscle action potential as a reliable marker in amyotrophic lateral sclerosis.

INTRODUCTION/AIMS: Reliable neurophysiological markers in amyotrophic lateral sclerosis (ALS) are of great interest. The compound muscle action potential (CMAP) amplitude has been a conventional marker, although it is greatly influenced by the electrode position. We propose the far-field potential of the CMAP (FFP-CMAP) as a new neurophysiological marker in ALS. METHODS: Patients with ALS and age-matched healthy controls were enrolled. We used a proximal reference (pref) in addition to the conventional distal reference (dref). Routine CMAP was recorded from the belly-dref lead and FFP-CMAP from the dref-pref lead for the ulnar and tibial nerves. Multiple point stimulation motor unit number estimation (MUNE) was also examined in the ulnar nerve. Inter-rater reproducibility was evaluated by two examiners, and some patients were followed up every 3 mo for 1 y. RESULTS: We tested 17 patients with ALS and 10 controls. The amplitudes of routine CMAP and FFP-CMAP in the ulnar and tibial nerves, and hypothenar MUNE value in the ulnar nerve were significantly decreased in ALS compared to controls. Ulnar FFP-CMAP achieved the highest inter-rater intraclass correlation coefficient (ICC) value (0.942) when compared with routine CMAP (0.880) and MUNE (0.839). The tibial FFP-CMAP had a higher ICC value (0.986) than the routine CMAP (0.697). In this way, the FFP-CMAP showed high inter-rater reproducibility because its shape was not much influenced by the electrode position. During 1-y follow-up, decline of CMAP, FFP, and MUNE showed significant correlations with the Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (ALSFRS-R). DISCUSSION: The FFP-CMAP shows promise as a reliable marker for ALS.

Preoperative findings in relation to the usefulness of endoscopic assistance for microvascular decompression.

PURPOSE: Endoscopy is known to be a useful adjunct for microvascular decompression (MVD) surgery, assisting observation in blind spots such as Meckel's cave in cases of trigeminal neuralgia (TN) and the root exit zone (REZ) in hemifacial spasm (HFS). However, few reports have discussed the usefulness of endoscopy in relation to individual patient characteristics or preoperative magnetic resonance imaging (MRI). METHODS: We retrospectively reviewed the medical records of 109 patients (98 with HFS and 11 with TN, 85 women, median age 55 years) who had undergone endoscopically assisted MVD at our institution between 2017 and 2021. The usefulness of endoscopy in individual cases was scored by three neurosurgeons using a grading scale: 2, essential and indispensable; 1, useful and helpful; 0, not necessary. The mean value of the assigned scores was taken as an indicator of "usefulness," and endoscopy was considered to have been "useful" in cases with a score of > 1.0. RESULTS: Endoscopic assistance was judged to have been useful in 69% of the patients. The proportion of patients evaluated as useful was significantly lower for TN (18.2%) than for HFS (74.5%). Patients with superior cerebellar artery compression had significantly lower scores than patients with other vessels. Endoscopy was considered useful in a significantly higher proportion of patients with anatomically complicated offending vessels (bifurcation or strong meandering) in the REZ detected by preoperative MRI (P < 0.005). CONCLUSION: Endoscopy for MVD is useful for patients with HFS, especially when preoperative MRI shows bifurcation or strongly meandering vessels in the REZ.

[Technique of Microvascular Decompression for Hemifacial Spasm Based on Surgical Anatomy].

This article describes the anatomical approach and operative points of microvascular decompression for hemifacial spasm based on the author's personal experience with 1,000 cases. As vascular compression of the facial nerve is often quite caudal, it is necessary to ensure that the compressing arteries in this area are moved. Neither the vessels nor the prosthesis used to move them should not touch the root exit zone of the nerve(vascular transposition nerve isolation technique). It should also be emphasized that every effort should be made to avoid complications such as hearing impairment and postoperative subcutaneous spinal fluid accumulation.

A new method to define cutoff values in nerve conduction studies for carpal tunnel syndrome considering the presence of false-positive cases.

BACKGROUND: Nerve conduction studies (NCS) are useful tools for diagnosing carpal tunnel syndrome (CTS). Establishing the normal values is the first step required for utilizing NCS for diagnosis. Previous epidemiological studies demonstrated the presence of fairly large number of false-positive subjects regarding NCS among control population, which has not been properly considered in past studies. This study proposed a new method to address this issue. METHODS: Non-diabetic 144 CTS patients were retrospectively enrolled using clinically defined inclusion criteria. Controls consisted of 73 age-matched volunteers without hand symptoms. Six NCS parameters were evaluated including peak-latency difference by the thumb method (thumbdif) and that by the ring-finger method (ringdif). The Youden index of the receiver operator characteristic curve was used both to judge the sensitivity of a parameter and to identify false-positive cases that were thought to have subclinical median neuropathy at the wrist. The linear function of six parameters was constructed, and the coefficient for each parameter was variously changed. RESULTS: When the Youden index took on the maximum value, seven control subjects (10%) were identified as false-positive and were excluded from the calculation of normal values. The most sensitive parameter before exclusion was thumbdif, whereas ringdif became the most sensitive after exclusion. The cut-off value for ringdif was 1.15 ms before exclusion, but was 0.37 ms after exclusion. CONCLUSION: This method can be widely applied to solve the statistical problem when the gold standard is lacking, and the outside reference standard is not completely reliable.

Prognosis of severe carpal tunnel syndrome with absent compound muscle action potential.

INTRODUCTION: Opponensplasty is a surgical option for patients with severe carpal tunnel syndrome (CTS). We investigated prognostic factors of patients who lack a preoperative compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle to determine the necessity for single-stage opponensplasty. METHODS: We retrospectively enrolled 22 hands of 22 CTS patients. Prognostic factors considered were age, diabetes mellitus, the median sensory nerve action potential, distal motor latency of the second lumbrical (2L) CMAP (2L-DML), and its amplitude (2L-Amp). Postoperative APB-CMAP amplitude (post APB-Amp) at 12 months was used as the outcome measure. RESULTS: Only 2L-DML showed a significant correlation with post APB-Amp (r = -0.56). The contribution of 2L-Amp was not significant, although 3 hands with absent 2L-CMAP had a poor electrophysiological recovery. CONCLUSIONS: Prolonged 2L-DML and absent 2L-CMAP seem to be poor prognostic factors. Concurrent opponensplasty may not be necessary in patients with 2L-DML of 8 ms or less. Muscle Nerve 54: 427-431, 2016.

Does arteriosclerosis contribute to hemifacial spasm?

BACKGROUND: Hemifacial spasm (HFS) is caused by pulsative vascular compression of the root exit zone (REZ) of the facial nerve. However, the mechanism that causes the offending vessels to compress the REZ has not been clarified. Elongation of intracranial arteries due to arteriosclerosis is one possibility, but such arteriosclerotic changes are not observed very frequently among patients with HFS. The aim of the present study was to investigate whether arteriosclerotic changes would contribute to the pathogenesis of HFS. METHODS: This study included 111 HFS patients, all of whom were Japanese. The prevalence rates of hypertension, hyperlipidemia, and diabetes mellitus were examined as risk factors of atherosclerosis, and the cardio-ankle vascular index (CAVI) was measured as an indicator of arteriosclerotic change. The severity of white matter lesions (WMLs) in HFS patients was measured by magnetic resonance imaging. These data were compared with data from healthy Japanese controls. RESULTS: The prevalence rates of the risk factors for atherosclerosis in the HFS patients were not higher than those in the general Japanese population. The CAVI scores for the HFS patients were similar to, or lower than those in the healthy controls for all age groups except 60 to 69-year-old men. The severity of WMLs in the HFS patients was not significantly worse than that in the controls. CONCLUSIONS: It is suggested that arteriosclerotic changes are not involved in the pathogenesis of HFS, and that vascular compression syndromes are attributable to anatomical features of the intracranial arteries and facial nerves formed during the prenatal stage.

Repetitive transcranial magnetic stimulation once a week induces sustainable long-term relief of central poststroke pain.

OBJECTIVE: Central poststroke pain is a serious problem for some patients after stroke. Repetitive transcranial magnetic stimulation (rTMS) has been reported to relieve poststroke pain but its efficacy is still controversial. We tested the possibility that rTMS, when applied once a week, would induce sustainable relief of poststroke pain. MATERIALS AND METHODS: Eighteen patients with central poststroke pain were included in this study. rTMS (10 trains of 10-sec 5 Hz-rTMS) was delivered over the primary motor cortex on the affected side. The rTMS session was repeated once a week for 12 weeks, and for six patients the intervention was continued for one year. The degree of the pain was assessed before each weekly rTMS session to evaluate sustainable effects. RESULTS: The effects of the rTMS reached a plateau at the eighth week. At the 12th week, the rTMS was effective in 61.1% of the patients; 5 of the 18 patients showed more than 70% reduction based on a visual analog scale, 6 patients showed 40-69% reduction, and 7 remained at a pain reduction level of less than 40%. When patients were divided into two groups with or without severe dysesthesia, it was found that eight patients with severe dysesthesia showed less pain relief than those without. In the six patients who continued rTMS for one year, the pain relief effects also were sustained. CONCLUSION: Although this was an open-label study without a control group, our findings suggest that rTMS of the primary motor cortex, when maintained once a week, could help to relieve poststroke pain.

Preoperative assessment of hemifacial spasm by the coronal heavily T2-weighted MR cisternography.

BACKGROUND: Microvascular decompression (MVD) has become a well-established surgical procedure for hemifacial spasm (HFS). Before surgery, it is essential to evaluate any possible deformity of the brainstem and establish the precise location of the offending vessels. In the present study of HFS patients we examined coronal sections taken by heavily T2-weighted MR cisternography in addition to routine axial sections, and assessed the usefulness of these images through comparison with intraoperative findings. METHODS: Eighty patients with HFS underwent preoperative coronal heavily T2-weighted MR cisternography before microvascular decompression surgery. Three neurosurgeons examined the preoperative axial and coronal MR images and evaluated vessel invagination into the brainstem. The usefulness of coronal sections was assessed statistically by the Mann-Whitney U test. RESULTS: Invagination of the offending vessel into the brainstem was observed in 24 cases (30.0%). In 19 patients, it was predicted preoperatively that compression of the flocculus and brainstem would be required in order to approach the offending vessels. Coronal MR cisternography was significantly more useful in cases with vessel invagination into the brainstem than in cases without invagination. CONCLUSIONS: Coronal sections obtained by MR cisternography are able to demonstrate the severity of vessel invagination into the brainstem as well as revealing the presence of the offending vessel. This information is helpful for planning a suitable approach to the root exit zone.

Adult-onset Sacral Meningocele Causing a Specific Headache Triggered by Compression or Adoption of a Sitting or Supine Posture.

We report a rare case of adult-onset sacral meningocele where compression triggered a specific headache. A 46-year-old woman presented with a headache, which worsened when she was in a sitting or supine position. A subcutaneous mass was observed on her left buttock, the compression of which also induced headache. No neurological deficits were evident. Lumbar and sacral magnetic resonance imaging demonstrated a meningocele in the left dorsal buttock, connecting to the sacral cerebrospinal fluid (CSF) space, and spinal computed tomography revealed sacral dysplasia. Initial meningocele resection improved the patient's headache, but the cyst recurred 2 years later. Following repeated surgery to reinforce the meningocele orifice, the headache was relieved and has been absent for more than 6 years. The headache was due to intracranial pressure fluctuations due to CSF influx into and drainage from the meningocele. Meningocele development in adulthood can be owing to a spinal bone defect and pressure load on the spinal dura. Surgical resection can improve symptoms resulting from meningocele, and reinforcement of the orifice using an artificial surgical membrane effectively prevents recurrence.

Deep Brain Stimulation Leads to Long-term Improvement of Neuropathic Tremor due to Chronic Inflammatory Demyelinating Polyneuropathy: A Case Report.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a peripheral neuropathy caused by immune-mediated demyelination, causing tremors in 3.9%-58% of affected patients. This neuropathic tremor may persist after treatment and is known to be refractory to conventional medication. We present two cases of neuropathic tremor due to CIDP in which deep brain stimulation (DBS) over a long-term period led to marked improvement. Case 1: A 66-year-old woman presented with severe 2-3-Hz resting, postural, and kinetic tremors of both hands. The tremor was refractory to medication but improved well after bilateral VIM-DBS. However, 2 months after the procedure, the tremor worsened and was accompanied by sensory disturbance in the extremities. A diagnosis of CIDP was made, and treatment with corticosteroids and intravenous immunoglobulin achieved remission 6 months later. Although there was residual tremor after CIDP remission, it has been well controlled by DBS for the last 10 years. Case 2: A 56-year-old man presented with a 6-year history of CIDP after developing sensory dullness and tremors in the extremities. The CIDP had gone into remission 1 year previously and the sensory deficits had improved, but the tremors had gradually worsened: severe 8-12-Hz postural, kinetic, and resting tremors were present in both upper extremities. Right VIM-DBS was performed and the tremors on the left side showed marked improvement. Over the next 8 years, the tremors were well controlled and there were no relapses of CIDP. DBS may achieve long-term improvement of neuropathic tremor caused by CIDP if the CIDP is in remission.

A Case of Unilateral Facial Spasm With Vulnerable Hearing Function Due to a History of Cisplatin Treatment, Resulting in Intraoperative Hearing Loss.

A 51-year-old man with a history of cisplatin treatment for a right testicular tumor underwent microvascular decompression for hemifacial spasm. At an early stage in the surgical procedure, the intraoperative auditory brainstem response (ABR) was diminished despite a relatively minimally invasive approach, resulting in irreversible hearing loss. Cisplatin is known to cause dose-dependent hearing impairment primarily affecting the cochlea, but it can also induce neurotoxicity. In the present case, prior cisplatin administration may have caused fragility of the cochlear nerve as well. Patients with a history of ototoxic and neurotoxic drugs such as cisplatin require more careful manipulation and thorough intraoperative auditory monitoring during neurosurgical procedures that may affect hearing, such as those for hemifacial spasms.

Is Common Trunk Anomaly Frequent in Hemifacial Spasm? Comparison with Normal Subjects Using Magnetic Resonance Imaging.

OBJECTIVE: Hemifacial spasm (HFS) results from vascular compression at the root exit of the facial nerve. Although the underlying etiology has yet to be identified, it has been suggested that congenital vascular anomalies are involved. We have hypothesized common trunk anomaly of the anterior inferior cerebellar artery (AICA) and posterior inferior cerebellar artery (PICA) which may play a role in HFS. However, no previous studies have directly compared the incidence of this anomaly between HFS patients and normal subjects. The present study was designed to address this gap in the literature. METHODS: This case-control study compared magnetic resonance angiography data from 65 HFS patients and 65 normal subjects. Dominant AICA/PICA is defined as the absence of PICA/AICA, with the remaining AICA/PICA supplying both vascular territories. The term "common trunk" encompasses both dominant AICA and dominant PICA. The frequency of common trunk and dominant AICA/PICA was compared between the 2 groups. RESULTS: There were no significant differences in the incidence of a common trunk (68.5% and 64.6%), dominant AICA (30.8% and 32.3%) and dominant PICA (37.7% and 32.3%) between the 2 groups. Additionally, no differences were found in the frequency of atherosclerosis risk factors. CONCLUSIONS: There was no apparent association between common trunk anomaly and HFS. It is suspected that some vascular anomalies other than a common trunk are involved in HFS.

Utility of the Repetitive Nerve Stimulation Test and Needle EMG in the Trapezius Muscle for the Early Diagnosis of ALS.

OBJECTIVES: To document the utility of decremental responses in the repetitive nerve stimulation test (RNS) and spontaneous activities in needle electromyography (EMG) in the trapezius muscle for the diagnosis of amyotrophic lateral sclerosis. METHODS: Subjects were retrospectively identified from our EMG database. Cervical spondylosis was represented as a disease control group. We investigated the sensitivity and specificity of RNS and EMG in the trapezius muscle and those of diagnostic criteria including the Gold Coast criteria (GCC). RESULTS: We reviewed 120 patients with amyotrophic lateral sclerosis and 17 patients with cervical spondylosis. "RNS or EMG" achieved the highest sensitivity (85%). The specificity was the highest for RNS (94%). Addition of RNS of the deltoid muscle achieved 98% sensitivity in the upper-limb onset amyotrophic lateral sclerosis. The sensitivity of the GCC was very high (88%). CONCLUSIONS: Neurophysiological parameters investigated in this study having close to 100% specificities or sensitivities are useful as complements to the GCC.

[Tremor].

Tremor is defined as an involuntary, rhythmic oscillatory movement of a body part. Based on clinical symptoms, tremors are classified into rest, postural, simple kinetic, intention, and task-specific tremors. Pathological conditions associated with tremors include Parkinson's disease, essential tremor, cerebellar tremor, task-specific focal dystonia, peripheral neuropathy induced tremors, tremor secondary to poisoning/drugs, and physiological tremor. In this article, we discuss classifications and tremor etiologies, as well as diagnosis and treatments including surgical management.

Neuroprotective Effects of Artificial Cerebrospinal Fluid: Analysis of Brainstem Auditory-Evoked Potential Monitoring During Microvascular Decompression in 117 Consecutive Patients.

BACKGROUND AND OBJECTIVES: To study the efficacy of irrigation with artificial cerebrospinal fluid (aCSF) for protection of cranial nerves during surgery; the time required for recovery of brainstem auditory-evoked potentials (BAEPs) that would reflect cochlear function was analyzed in comparison with that for saline irrigation. METHODS: This retrospective study included 117 consecutive patients (95 women, mean age 51.5 ± 11.4 year) who underwent microvascular decompression for hemifacial spasm. During surgery, BAEPs were monitored to avoid damage to the auditory pathways. When a delayed latency of >1 ms or a decrease in amplitude of >50% was detected in BAEP wave V, surgical maneuvers were halted, and the operative field was irrigated with saline or aCSF. Saline was used for irrigation in 58 patients and aCSF in the other 59. The time required for BAEP recovery at the first halt in each patient was analyzed, and the results were compared between the groups. RESULTS: Surgical procedures were interrupted because of BAEP latency delays or decreases in amplitude in 51 of the patients in the saline group and 54 in the aCSF group. In both groups, the latencies and amplitudes recovered significantly with time and both recovered earlier after aCSF irrigation than after saline irrigation. Hearing outcome was not significantly different between 2 groups. CONCLUSION: aCSF is effective for protection of cochlear nerve and promotes recovery from transient dysfunction during surgery. The protective effect may be attributed to multiple factors including conditioned pH, electrolyte composition, glucose, and microelements, such as magnesium and phosphate.

Regression of Acoustic Tumor After Chemotherapy for Ovarian Cancer in a Patient With a Breast Cancer Susceptibility Gene 1 (BRCA1) Germline Mutation.

We report the case of an adult woman who developed ovarian cancer during a follow-up for vestibular schwannoma. Volume reduction of the schwannoma was observed after chemotherapy for ovarian cancer. After ovarian cancer had been diagnosed, the patient was found to have a germline mutation of breast cancer susceptibility gene 1 (BRCA1). This is the first reported case of vestibular schwannoma in a patient with a germline mutation of BRCA1 and the first documented example of chemotherapy including olaparib to have shown efficacy for schwannoma.

Bilateral Low-Frequency Hearing Impairment After Microvascular Decompression Surgery.

BACKGROUND: Hearing impairment is an important complication of microvascular decompression (MVD). In patients after MVD, we have occasionally noted slight to moderate hearing deterioration at low frequencies that is difficult to detect using pure tone average. OBJECTIVE: To assess the incidence and features of low-frequency hearing impairment (LF-HI) after MVD and evaluate its associated factors. METHODS: This single-center, retrospective observational study assessed the audiometric outcome of 270 patients who underwent MVD between January 2015 and December 2020. Preoperative and postoperative hearing levels were compared for each frequency. LF-HI was defined as a hearing deterioration of ≥15 dB at 125, 250, or 500 Hz. The incidence, symptoms, and associated factors of LF-HI were analyzed. RESULTS: Statistical analysis of the patients overall demonstrated slight but significant decreases in the hearing level after MVD at lower frequencies on both the operative and contralateral sides. Eighty-one patients (30.0%) had LF-HI: 49 on the operative side, 24 on the contralateral side, and 8 on both sides, while pure tone average was worsened in 5 patients (1.8%). Subjective symptoms, including hearing deterioration, ear fullness, tinnitus, and dizziness, developed in 10.4% of the patients with LF-HI but improved subsequently within several weeks. "Older age" and "operative side" were associated with LF-HI. CONCLUSION: Decreases in lower-frequency hearing levels in both the ipsilateral and contralateral (nonoperative) ears were observed after trigeminal neuralgia and hemifacial spasm surgery. LF-HI does not cause permanent symptoms but may be a noteworthy phenomenon, possibly involved in the contralateral hearing loss encountered occasionally after other types of posterior cranial fossa surgery.

Aspiration Surgery with Appropriate Antibiotic Treatment Yields Favorable Outcomes for Bacterial Brain Abscess.

BACKGROUND: Even in the era of advanced medical treatment, brain abscess still has a high mortality rate. At our institution, brain abscess has been treated mainly using stereotactic or echo-guided aspiration followed by relatively long-term antibiotic treatment, achieving favorable outcomes. To evaluate the efficacy of our strategy involving less-invasive aspiration surgery and long-term selective antibiotic administration for brain abscess, a single-institution series of cases was investigated. METHODS: We retrospectively reviewed and analyzed the medical records of 25 cases of brain abscess treated at Saitama Medical University Hospital between 2008 and 2021. The patients comprised 16 men and 9 women aged between 39 and 85 years (median 62 years). Neurosurgical intervention was performed for 23 (92.0%) of the patients and the remaining 2 received antibiotics alone. RESULTS: Among the neurosurgery patients, 22 (95.7%) underwent echo-guided or stereotactic aspiration, and only 1 underwent craniotomy. Anaerobic bacteria were detected in 11 patients. In the surgical and conservative groups, the median duration of antibiotic treatment was 16 weeks and 23 weeks, respectively. Since 2014 when metronidazole first became available, it has replaced meropenem to cover anaerobic bacteria. The overall mortality rate was 4.0% and a favorable outcome (Glasgow Outcome Scale 4 or 5) was achieved in 76% of the patients. There was no surgical mortality or morbidity. CONCLUSIONS: Most patients underwent aspiration surgery and achieved favorable outcomes. Along with antibiotic treatment for a sufficiently long period to cover anaerobes, this approach can be expected to yield good results.