Seizures may occur after stroke. Though the majority of clinicians are aware of this, a consensus-based treatment and management strategy for post-stroke seizures is not available because there have only been a few large-scale studies that have explored this. This study has surveyed the actual state of medical treatment for post-stroke seizure and epilepsy in Japan. We conducted a nationwide questionnaire survey of the top 500 institutions regarding the number of cerebral infarction cases between February 2015 and May 2015. The questionnaire contained 14 items regarding the number of patients, type of tests and treatments conducted, and patient response to the treatments. Surveys from 189 institutions were obtained. A history of previous stroke was reported in 41% of hospitalized patients with epilepsy. The sensitivity of diffusion-weighted MRI and electroencephalography was not sufficient to detect the abnormalities seen in epilepsy. Carbamazepine was the most chosen antiepileptic drug for secondary prophylaxis, followed by valproate acid, and levetiracetam.
A 63-year-old man developed a syndrome of cauda equine, with the numbness which is a left lower extremity from the left buttocks, weakness of left leg, and a dysfunction of bladder and bowel. Enhanced MRI revealed the enhancement of lower cauda equine, and a nerve conduction test revealed decreased F-wave persistency in the tibial nerve and increased F-wave latency in the peroneal nerve on the both sides. M-proteinemia was admitted and myeloma was suspected. By a biopsy of a vertebral arch, we diagnosed with diffuse large B-cell lymphoma. We treated with dexamethasone and R-CHOP (rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone (prednisolone)), then the symptom was improved. In case of caude equine syndrome with M-proteinemia, a possibility of the malignant lymphoma should also be considered.
A 25-year-old woman in her 37 weeks and 5 days pregnant presented with abnormal behavior and memory impairment following a high fever. Her manifestations were diagnosed as limbic encephalitis, and she delivered a baby by Cesarean section. In the operation, bilateral ovarian tumors were found and resected, though they were revealed as non-teratoma afterward. After operation, she became agitated, and started to present oral dyskinesia, intractable systemic myoclonus, central hypoventilation, and autonomic manifestations such as labile blood pressure, but her symptoms responded well to immunotherapy. Her clinical course was typical for anti-N-methyl D-aspartate (NMDA) receptor encephalitis, but anti-NMDA receptor antibody was not detected in her serum and CSF. On the other hand, anti SS-A antibody was positive in her serum, and the lip biopsy findings confirmed Sjögren’s syndrome (SjS). Only several cases of SjS-associated limbic encephalitis have been reported, but none of them mimicked anti-NMDA receptor encephalitis. This patient indicates that SjS should be considered even in a case of limbic encephalitis with a typical clinical spectrum of anti-NMDAR encephalitis.
A 55-year-old man with no history of stroke or migraine presented to the clinic with cognitive impairment and depression that had been experiencing for two years. Neurological examination showed bilateral pyramidal signs, and impairments in cognition and attention. Brain MRI revealed multiple lacunar lesions and microbleeds in the deep cerebral white matter, subcortical regions, and brainstem, as well as diffuse white matter hyperintensities without anterior temporal pole involvement. Cerebral single-photon emission computed tomography (SPECT) revealed bilateral hypoperfusion in the basal ganglia. Gene analysis revealed an arginine-to-proline missense mutation in the NOTCH3 gene at codon 75. The patient was administered lomerizine (10 mg/day), but the patient’s cognitive impairment and cerebral atrophy continued to worsen. Follow-up testing with MRI three years after his initial diagnosis revealed similar lacunar infarctions, cerebral microbleeds, and diffuse white matter hyperintensities to those observed three years earlier. However, MRI scans revealed signs of increased cerebral blood flow. Together, these findings suggest that the patient’s cognitive impairments may have been caused by pathogenesis in the cerebral cortex.
A 41-year-old man left for Mexico in May 2015. Right pulmonary nodule was detected at a health examination in May 2016, and he subsequently showed headache and slight fever. Contrast-enhanced magnetic resonance imaging of the brain revealed basilar meningitis, so he was admitted to our hospital. We considered imported mycosis due to his travel history to Mexico. We diagnosed histoplasmosis based on the presence of antibodies against Histoplasma in both serum and cerebrospinal fluid. Symptoms almost completely recovered with a liposomal formulation of amphotericin B. Central nervous system histoplasmosis is very rare in Japan. Immunocompetent hosts can develop histoplasmosis, and this pathology is important to consider in patients presenting with basilar meningitis and a positive travel history.
A 75-year-old man having dementia and lifestyle related diseases developed a lobar intracerebral hemorrhage (LICH) in the left parietal and a small cerebellar infarction in the left occipital lobe. Many micro bleeds (MB) due to cerebral amyloid angiopathy (CAA) in the subcortical areas and multiple vascular stenosis were also found by MRI and MRA. He developed herpes zoster in his buttocks on day 6 of hospitalization and complicated with varicella zoster virus (VZV) meningitis with positive for VZV-DNA in the cerebrospinal fluid. Subsequently, LICHs occurred in the left frontal lobe and in the right parietal lobe for a short period of time and died on the day 18. We speculated that the repeating hemorrhages was primarily caused by VZV vasculopathy and additionally the subcortical MBs increased the hemorrhagic risk. The relationship between VZV vasculopathy and CAA should be studied in the future.