Delayed blink R1 latency in a patient with trigeminal neuralgia due to a contralateral vestibular schwannoma: An illustrative case.

Background: Although the blink reflex (BR) is effective in objectively evaluating trigeminal neuropathy, few studies have demonstrated its effect on trigeminal neuralgia (TN). The authors report a patient with TN due to contralateral vestibular schwannoma (VS) functionally diagnosed by delayed R1 latency of the BR. Case Description: A 36-year-old man presented with left-sided deafness and paroxysmal facial pain in the right V1-3 area. Magnetic resonance imaging (MRI) showed a solid cystic mass compressing the right pons and left brainstem at the left cerebellopontine angle. Although preoperative BR evoked by right supraorbital nerve stimulation-induced delayed ipsilateral R1 latency and normal ipsilateral and contralateral R2 responses, the BR latency evoked by left supraorbital nerve stimulation was normal, indicating deficits in the principal nucleus of the trigeminal nerve in the right pons. The symptoms of TN disappeared after the removal of the VS. Postoperative MRI showed subtotal removal of the tumor and sufficient decompression of the pons and cerebellopontine cistern. The R1 latency returned to normal 50 days after surgery. Conclusion: The perioperative BR test was not only useful for objective evaluation of the localization of trigeminal neuropathy but also correlated with the symptoms of TN.

Camurati-Engelmann disease combined with transethmoidal meningoencephalocele: illustrative case.

BACKGROUND: Camurati-Engelmann disease (CED) is a rare disorder characterized by progressive cranial hyperostosis and diaphyseal sclerosis of the long bones. Chronic intracranial hypertension gradually occurs due to progressive cranial vault hyperostosis. OBSERVATIONS: A 57-year-old man who had been diagnosed with CED at 9 years old suddenly developed cerebrospinal fluid rhinorrhea. A bone defect of the right cribriform plate and protrusion of brain tissue from the right cribriform plate into the right nasal cavity were identified. The patient underwent endoscopic resection of the meningoencephalocele combined with the bath-plug procedure. After surgery, cerebrospinal fluid rhinorrhea disappeared. LESSONS: Chronic intracranial hypertension due to progressive cranial vault hyperostosis in CED may cause a bone defect and meningoencephalocele in the anterior skull base, resulting in cerebrospinal fluid rhinorrhea.

Transfemoral subclavian artery stenting through a shaped guiding catheter without pull-through technique: A case report.

Transfemoral subclavian artery stenting can be challenging unless the placement of the guiding catheter is secured. Herein, we present a patient with subclavian artery stenosis treated with endovascular stenting using a shaped guiding catheter. A 79-year-old woman was admitted to our department because of a cold sensation and numbness of her left arm. Computed tomography revealed stenosis of the left subclavian artery (SA), located just proximal to the ostium of the left vertebral artery (VA). Doppler ultrasound showed reverse flow in the left VA. We planned to stent for the SA stenosis under the balloon protection of the left VA. The balloon protection device was easily navigated into the left VA through brachial access. After that, a self-expandable stent was successfully placed from just proximal to the VA origin to the ostium of the SA using a highly stable shaped guiding catheter. The patient recovered from the symptoms and was discharged 4 days after the procedure. The high stability of the shaped guiding catheter is advantageous during endovascular treatment of the subclavian artery.

Cerebral protection during retrograde brachiocephalic artery stenting using a single filter and increased subclavian steal phenomenon: illustrative case.

BACKGROUND: Cerebral protection during brachiocephalic artery (BCA) stenting is important. However, the maneuver is sometimes challenging because both the internal carotid artery (ICA) and vertebral artery (VA) should be protected. Herein, the authors present an alternative cerebral protection technique involving filter protection for the ICA and hemodynamic protection for the VA during retrograde BCA stenting. OBSERVATIONS: A 64-year-old man with a thoracic aortic aneurysm presented with cold sensation and numbness in his right arm due to BCA stenosis. Endovascular stenting under cerebral protection was planned. Cerebral protection was attempted through the brachial access. Despite the successful placement of the filter in the ICA, selective catheterization of the VA failed. Furthermore, repeated transfemoral catheterization of the BCA was unsuccessful. Concerning a thoracic aortic aneurysm injury, the authors performed retrograde BCA stenting using a transbrachial approach. Hemodynamic protection of the VA was provided by increasing the subclavian steal phenomenon that resulted in successful recanalization of the BCA. LESSONS: Retrograde BCA stenting performed while protecting the ICA with a filter and the right VA by increasing the subclavian steal phenomenon was successful. This simple technique is feasible, especially in patients with steno-occlusive lesions of the BCA concurrent with the dominant vertebra/vertebral collateral pathway.

Detection of impaired cerebrovascular reactivity in patients with chronic cerebral ischemia using whole-brain 7T MRA.

BACKGROUND: Cerebrovascular reactivity (CVR) to acetazolamide (ACZ) on single-photon emission computed tomography (SPECT) can be used to assess the severity of chronic cerebral ischemia; however, this is an invasive method. We examined whether whole-brain magnetic resonance angiography (MRA) at 7T could non-invasively detect impaired CVR in patients with chronic cerebral ischemia by demonstrating the leptomeningeal collaterals (LMCs). METHODS: Fifty-seven patients with symptomatic unilateral cervical stenosis underwent whole-brain time-of-flight MRA at 7T and cerebral perfusion SPECT before/after the ACZ challenge. MRA images were visually assessed based on 6-point grading systems to evaluate the development of LMCs toward the middle cerebral artery (MCA) and antegrade flow of MCA. CVR of the affected side was calculated from the SPECT data. Subsequently, we compared the LMC grades on MRA with CVR on SPECT. RESULTS: CVR was significantly lower in grades ≥ 2 of LMCs than in grades 0-1 (P < 0.05) when applying LMCs from the anterior cerebral artery (ACA) and/or posterior cerebral artery (PCA). These differences were more evident than those in the grading of the antegrade MCA flow. The LMC grades from ACA/PCA readily detected reduced CVR (< 18.4%) with a sensitivity/specificity of 0.79/0.82. CONCLUSION: The development of LMCs on whole-brain MRA at 7T can non-invasively detect reduced CVR with a high sensitivity/specificity in patients with unilateral cervical stenosis.

Primary Plasmacytoma in the Cerebellum: A Case Report and Literature Review.

BACKGROUND: Most plasmacytomas arise in the bone marrow (intramedullary), as part of multiple myeloma (MM). In contrast, extramedullary plasmacytoma without MM is rare, and plasmacytoma primarily occurring in the brain parenchyma is extremely rare. Clinical behaviors of primary plasmacytoma in the brain have remained unclear. We report a case of primary plasmacytoma in the cerebellum and review the literature. CASE DESCRIPTION: The patient was a 33-year-old woman, displaying vertigo and peripheral facial nerve palsy. A tumor was identified in the subcortical white matter of the middle-upper cerebellum. Magnetic resonance imaging showed no specific findings for this lesion. Tumor was surgically resected because of aggressive tumor growth. Pathologic diagnosis of the tumor was plasmacytoma. The patient was treated with irradiation to the tumor bed after surgery. Although histology of the bone marrow showed a few atypical plasma cells (1%-2%), below the threshold of the diagnostic criterion for MM, we started chemotherapy to prevent occurrence of MM. Neither tumor recurrence nor development of MM was found for 16 months after surgery. CONCLUSIONS: Histology is essential for diagnosis of primary plasmacytoma in the brain because of the lack of specific findings on neuroimaging. A finding of a few atypical plasma cells in the bone marrow might support the assumption that extramedullary plasmacytoma represents a harbinger of subsequent development of MM. In addition to appropriate therapies combining maximum tumor removal and radiotherapy to the brain, rigorous hematological management might have contributed to favorable outcomes.

Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic moyamoya disease: subanalysis of a prospective cohort.

OBJECTIVE: Adult patients with ischemic moyamoya disease (MMD) who receive treatment with antiplatelet drugs reportedly show improvements in neuropsychological test scores after around 2 years. The purpose of the present study subanalyzing the same patient cohort used in a previous study was to determine which antiplatelet drug, clopidogrel or cilostazol, results in better improvement of cognitive function among non-surgical adult patients showing ischemic MMD without severe hemodynamic compromise. METHODS: Sixty-six patients without cerebral misery perfusion on 15O gas positron emission tomography were treated with pharmacotherapy alone. Patients ≥50 years old and <50 years old initially received clopidogrel and cilostazol, respectively. Any patient suffering side effects of the antiplatelet drug switched to the other antiplatelet drug. Neuropsychological tests were performed at study entry and at the end of the 2-year follow-up, and differences in each neuropsychological test score between the two time points (second test score - first test score) were calculated and defined as Δ scores. RESULTS: Among the five neuropsychological tests, Δ scores for two tests were significantly greater in patients treated with cilostazol (n = 36) than in those treated with clopidogrel (n = 30), and Δ scores of the remaining three tests did not differ between patient groups. Based on Δ scores, 15 patients (23%) were defined as showing interval cognitive improvement. On multivariate analysis, cilostazol administration (95% confidence interval, 1.19-193.98; P = 0.0361) represented an independent predictor of interval cognitive improvement. CONCLUSIONS: Cilostazol may improve cognition better than clopidogrel in non-surgical adult patients with ischemic MMD.

Impact of cerebral blood flow changes due to arterial bypass surgery on cognitive function in adult patients with symptomatic ischemic moyamoya disease.

OBJECTIVE: Some adult patients with moyamoya disease (MMD) undergoing revascularization surgery show an improvement or decline in cognition postoperatively. Revascularization surgery for ischemic MMD augments cerebral blood flow (CBF) and improves cerebral oxygen metabolism. However, cerebral hyperperfusion, which is a short-term, major increase in ipsilateral CBF that is much greater than the metabolic needs of the brain, sometimes occurs as a complication. Cerebral hyperperfusion produces widespread, minimal injury to the ipsilateral white matter and cortical regions. The aim of the present prospective study was to determine how changes in CBF due to arterial bypass surgery affect cognitive function in adult patients with symptomatic ischemic MMD and misery perfusion. METHODS: Thirty-two patients with cerebral misery perfusion, as determined on the basis of 15O gas positron emission tomography, underwent single superficial temporal artery-middle cerebral artery (M4 in the precentral region) anastomosis. Brain perfusion single-photon emission computed tomography (SPECT) studies were performed preoperatively, on the 1st postoperative day, and 2 months after surgery. Neuropsychological tests were also performed preoperatively and 2 months after surgery. RESULTS: Postoperative neuropsychological assessments demonstrated cognitive improvement in 10 cases (31%), no change in 8 cases (25%), and decline in 14 cases (44%). Based on brain perfusion SPECT and symptoms, 10 patients were considered to have cerebral hyperperfusion syndrome, and all of these patients exhibited a postoperative decline in cognition. Relative precentral CBF on the 1st postoperative day was significantly greater in patients with postoperative cognitive decline (167.3% ± 15.3%) than in those with improved (105.3% ± 18.2%; p < 0.0001) or unchanged (131.4% ± 32.1%; p = 0.0029) cognition. The difference between relative precentral CBF 2 months after surgery and that before surgery was significantly greater in patients with postoperative cognitive improvement (17.2% ± 3.8%) than in those with no postoperative change (10.1% ± 2.4%; p = 0.0003) or with postoperative decline (11.5% ± 3.2%; p = 0.0009) in cognition. CONCLUSIONS: Cerebral hyperperfusion in the acute stage after arterial bypass surgery impairs cognitive function. An increase in CBF in the chronic stage without acute-stage cerebral hyperperfusion improves cognitive function in adult patients with symptomatic ischemic MMD and misery perfusion.