Hypoglossal Nerve Palsy due to Compression by a Persistent Primitive Hypoglossal Artery: Case Report.

A 51-year-old Japanese woman was admitted to our hospital because of speech difficulty following severe headache. Neurological examination showed dysarthria and tongue weakness on the right side, indicating right hypoglossal nerve palsy. Needle electromyography of the right side of the tongue showed fibrillation potentials. Magnetic resonance angiography and computed tomography angiography revealed a right, persistent, primitive hypoglossal artery (PPHA) that met Lie's diagnostic criteria. Digital subtraction angiography showed an extended PPHA with irregular caliber in the portion running through the right hypoglossal canal. We diagnosed compression neuropathy of the hypoglossal nerve due to PPHA enlargement based on the findings of ipsilateral hypoglossal nerve palsy, fibrillation that indicated peripheral nerve palsy, and the enlarged diameter of the portion of the PPHA running through the right hypoglossal canal. We prescribed antihypertensive therapy. At 1 year after onset, her tongue weakness was alleviated. Clinicians should consider compression neuropathy due to a PPHA as one of the possibilities in the differential diagnosis of hypoglossal nerve palsy.

Helicopter Transport for Patients with Cerebral Infarction in Rural Japan.

OBJECTIVES: Physician-staffed helicopter transport is faster than ground transport and allows for prompt medical care of patients in rural areas. In this study, we evaluated the relationship between helicopter transport and the prognosis of patients with acute cerebral infarction in rural Japan. METHODS: This retrospective, observational study included 546 patients with acute cerebral infarction attending Aomori Prefectural Central Hospital, which serves a rural region of Japan. Patients were separated into 2 transport groups: physician-staffed helicopter emergency medical services and ground emergency medical services. Patients were assessed for stroke severity, treatment, and prognosis. RESULTS: Of the 546 patients, 11.2% were transported by physician-staffed helicopter emergency medical services and 88.8% by ground emergency medical services. Although the distance transported was significantly longer in the physician-staffed helicopter emergency medical services group, the time from onset to reaching our hospital was similar between the groups. National Institutes of Health Stroke Scale on admission and final prognosis were significantly worse with physician-staffed helicopter emergency medical services than with ground emergency medical services. Multivariate analysis showed no association between transport system and prognosis. CONCLUSIONS: In this study, patients transported by physician-staffed helicopter emergency medical services had more severe stroke symptoms and poorer functional outcomes than those transported by ground emergency medical services. However, the transport time was shorter for physician-staffed helicopter emergency medical services; thus, physician-staffed helicopter emergency medical services may be useful for reducing transport time for patients in rural Japan.

Subacute Sensory Ataxic Neuronopathy With Thymoma Presenting Marked Improvement After Steroid Therapy.

Subacute sensory ataxic neuronopathy is a well-known form of paraneoplastic syndrome. Most sensory neuronopathies are associated with small cell lung cancer and anti-Hu antibodies, and usually show only slight improvement with immunotherapy. To date, there have been few reports of neuropathy associated with thymoma and no treatment strategy has been established for thymoma-related neuropathy. Here, we provide the first report of a case of sensory ataxic neuronopathy with thymoma that showed marked improvement after steroid therapy, even though preceding intravenous immunoglobulin treatments and tumor resection were less effective. A 57-year-old Japanese man was referred to our hospital with a 6-week history of distal paresthesia in his four limbs and an unsteady gait. He presented with left-dominant ataxia in his four limbs due to reduced sensation in his extremities. He also complained of constipation, difficulty urinating, and erectile dysfunction. Upon investigation, including electrodiagnostic studies, the patient was diagnosed as having sensory ataxic neuronopathy with invasive thymoma. A first round of intravenous immunoglobulin therapy, a following thymectomy, and a second round of intravenous immunoglobulin therapy after the surgery were not effective in treating his neurological symptoms. Subsequently, oral steroid therapy was started, which brought about a remarkable improvement; 6 weeks after the beginning of the steroid therapy, his neurological symptoms were resolved, except for slight distal paresthesia in his feet. Although rarely reported, thymoma can underlie sensory neuronopathy, and the response of thymoma-associated sensory neuronopathy to immunotherapy might be better than that of anti-Hu antibody-related neuropathies. Even if the first immunotherapy is not effective in treating neuropathy with thymoma, further immunomodulatory treatment should be tried after treating the tumor.

Rectus Sheath Hematoma Following Intravenous Thrombolysis With Recombinant Tissue Plasminogen Activator for Cerebral Infarction: A Case Report.

A 76-year-old Japanese man was admitted to our department because of aphasia and right hemiparesis. Brain magnetic resonance imaging showed cerebral infarction caused by occlusion of the left middle cerebral artery, and alteplase therapy was started. Thrombectomy was subsequently performed, resulting in recanalization. On day 4 after admission, he complained of epigastric pain when coughing. On day 8, contrast-enhanced computed tomography showed a left abdominal rectus sheath hematoma without extravasation. We carefully followed up the patient with conservative therapy, and the rectus sheath hematoma was ameliorated. Rectus sheath hematomas present as acute abdominal pain and are often misdiagnosed. Although several predisposing factors for rectus sheath hematomas have been identified, whether recombinant tissue-type plasminogen activator causes rectus sheath hematomas, remains unclear. This case highlights the need to consider a rectus sheath hematoma as a differential diagnosis of abdominal pain following treatment with recombinant tissue-type plasminogen activator.

Association of survival and hyperthermia after rt-PA for ischemic stroke.

BACKGROUND: Hyperthermia in patients with acute ischemic stroke is associated with poor outcome. Although previous studies have shown a negative effect on functional outcome, even in patients treated with intravenous recombinant tissue plasminogen activator (rt-PA), the effect on survival remains unclear. AIMS OF THE STUDY: The aim of this study was to evaluate the association between the functional and survival prognosis and hyperthermia in patients with acute ischemic stroke treated with rt-PA. METHODS: We studied 120 patients treated with rt-PA from 2306 consecutive Japanese patients with acute cerebral infarction at Aomori Prefectural Central Hospital between December 2009 and March 2017. We defined hyperthermia as ≥38°C within 72 hours after rt-PA administration. Propensity score matching was used to compare 34 non-hyperthermia and hyperthermia patient pairs. RESULTS: Final modified Rankin Scale scores were higher in the hyperthermia group than in the non-hyperthermia group. In addition, the Kaplan-Meier model showed that the non-hyperthermia group had significantly better survival rates than the hyperthermia group (hazard ratio, 5.3; 95% confidence intervals, 1.2-24.8). CONCLUSIONS: Hyperthermia within 3 days after rt-PA is associated with poor functional prognosis and survival outcome in patients with acute cerebral infarction.

Myelin Oligodendrocyte Glycoprotein-IgG-positive Recurrent Bilateral Optic Papillitis with Serous Retinal Detachment.

Autoantibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have been detected in inflammatory demyelinating central nervous system diseases. A 30-year-old woman had blurred vision, marked optic nerve disc swelling, serous retinal detachment at the macular on optic coherence tomography, and MOG-IgG seropositivity. The patient was thought to have optic papillitis associated with MOG-IgG. Her symptoms rapidly improved after high-dose methylprednisolone therapy. We hypothesize that serous retinal detachment was secondary, arising from optic papillitis. This is the first report of the concurrence of optic papillitis with MOG-IgG and serous retinal detachment. MOG-IgG should be tested in patients with marked optic disc swelling.