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.

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.

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.