Rapid adaption of endovascular thrombectomy (EVT) is essential for patients with large arterial occlusion (LAO). Although patients transferred need longer transportation, they have an advantage of preadmission diagnosis regarding arterial occlusion. The aim of the present study is to evaluate whether optimizing the assessment at comprehensive center for patients transferred may improve the clinical outcome after EVT. Data on consecutive patients treated with EVT between September 2014 and May 2017 were studied. Generally, we have two distinct protocols for EVT candidates: 1) the transfer group, patients are directly taken to the CT and escorted to the angiography room; and 2) the direct group, patients receive the routine emergent evaluation and examined with MRI/MRA. Good outcome was defined as modified Rankin Scale score ≤1 at 3 months. Thirty-one (29%) patients were classified into the transfer group and the 77 (71%) were into the direct group. Although the onset to door time was longer in the transfer group (175 [137–275] min. vs. 76 [51–260] min, P = 0.001), the rate of good outcome was similar between the 2 groups (41% vs. 25%, P = 0.205). By multivariate regression analysis, the onset to reperfusion time was the independent factor (odds ratio 0.982, 95%CI: 0.967–0.998, P = 0.026) associated with good outcome, while transfer itself was not the independent parameter (odds ratio 0.732, 95%CI: 0.125–4.291, P = 0.730). Regarding time parameters, door to picture time (11 [7–24] min vs. 27 [21–39] min., P < 0.001) and picture to puncture time (27 [18–60] min. vs. 54 [39–78] min, P < 0.001) were shorter in the transfer group. Thus, the onset to puncture time (234 [177–299] min. vs. 170 [125–367] min, P = 0.063) and the onset to reperfusion time (271 [208–352] min. vs. 237 [159–382] min., P = 0.183) were similar between the 2 groups. Shortening the initial evaluation at comprehensive stroke center can provide a good outcome for patients transferred.

A 23-year-old man experienced numbness in the perioral region and right arm, and right leg weakness on the second day after drinking a large amount of alcohol during foreign travel. His symptoms disappeared but then reappeared repetitively. Cerebral MRI performed on the third day after onset showed multiple white matter lesions; however, these lesions disappeared 26 days after onset. Neurological examination and nerve conduction studies revealed demyelinating polyneuropathy. Genetic testing for Charcot-Marie-Tooth disease, X-linked dominant 1 (CMTX1) due to GJB1 mutation was conducted based on the symptoms of transient central nervous system lesions and polyneuropathy exhibited by the patient and his mother. As a result, a c.530T>C (p.V177A) substitution in exon 2 of GJB1 was identified. CMTX1 patients should be advised to avoid excessive drinking because this could induce central nervous system lesions.

We report on a 52-year-old woman with rheumatoid arthritis (RA) who developed methotrexate associated lymphoproliferative disorders (MTX-LPD) in the central nervous system (CNS) in the course of immunosuppressive therapy for RA. The patient was admitted because of monoplegia in her left hand. She had been receiving methotrexate (MTX) for her RA for several years and etanercept had also been introduced because of a worsening of the arthritis six months before admission. Brain MRI revealed multiple lesions with enhancement scattered throughout both hemispheres. ¹⁸F-fluorodeoxyglucose-positron emission tomography/computed tomography showed abnormal accumulation suggesting malignancy in the right frontal lobe where abnormal enhancement was observed on the MRI. A brain biopsy was performed at the identified site and it confirmed diffuse large B-cell lymphoma (DLBCL). We therefore diagnosed her as MTX-LPD. According to previous reports, most MTX-LPD cases tend to show regression after the cessation of MTX. However, our case showed no regression and even needed chemotherapy. The patient had a poorer prognosis than previous cases and died 17 months after the onset. Although it is an uncommon complication, particularly in the CNS, MTX-LPD should be considered as a critical differential diagnosis if a patient receiving MTX develops central nervous system lesions. Immediate medical intervention including brain biopsy is required.

A 69-year-old female with an old infarct of the left parietotemporal lobe was admitted for the evaluation of suspected painful seizures accompanying ictal paresis. The painful seizure and ictal paresis involved her right extremities without convulsions, although intermittent tremulous movements were noted on the right upper extremity. She also showed right hemianopia during the seizure. Ictal scalp EEG demonstrated lateralized rhythmic sharply contoured delta activity intermingled with a large amount of spikes, sharp waves, and fast activity mainly on the posterior half of the left hemisphere. Ictal MRI showed restricted diffusion in the postcentral gyrus and dilatation of distal branches of the left middle cerebral artery (MCA). ^99mTc-ECD SPECT revealed hyperperfusion on the left parietal cortex. Treatment with antiepileptic drugs successfully prevented seizure recurrence, then she was discharged home. On the follow-up SPECT after 1 month, the abnormal hyperperfusion disappeared. MRI demonstrated resolution of the restricted diffusion and the MCA dilatation. Taken together with the EEG abnormality and the transient abnormalities in SPECT and MRI, we concluded that her seizures were epileptic and that her painful seizures likely arise from the left primary somatosensory cortex. The mechanism of ictal paresis would be attributed to some disturbed functional architecture in the left primary motor cortex leading to loss of normal motor function through epileptic interference by ictal discharges. The same mechanism in the visual cortex could be assumed for her ictal hemianopia. Painful seizure and ictal paresis each is rarely encountered, even more so the combination thereof. These ictal manifestations might be difficult to differentiate from transient ischemic attack or postictal paresis, and thus EEG is essential to diagnose this treatable condition.

A 51-year-old man with a cerebral lacunar infarction of the midbrain that had occurred two years before, was transferred from a regional psychiatric hospital with chronic progressive psychiatric symptoms including cognitive decline, irritability and hallucinations. Neurological examinations upon admission revealed cerebellar ataxia including dysarthria, ataxic gait and bilateral intention tremor. Brain FLAIR MRI on day 2 revealed abnormal hyperintense lesions in the bilateral insular cortex and temporal pole. Treponemal and non-treponemal specific antibodies were positive in both serum and cerebrospinal fluid (CSF), indicating a diagnosis of neurosyphilis. Treatment with intravenous penicillin (24 × 10⁶ units/day × 28 days) improved his psychiatric symptoms, ataxia, imaging abnormalities and inflammatory CSF findings. Cerebellar ataxia is a rare symptom of neurosyphilis. Nonetheless, the possibility of neurosyphilis should be considered if a young adult ataxia accompanied by psychiatric symptoms.

A 63-year-old woman presented to our hospital with sudden symptoms of unsteadiness while walking. Based on the neurological findings, i.e., ataxia and absence of tendon reflex in the extremities accompanied by antecedent infection at the time, she was tentatively diagnosed with Fisher syndrome. Following intravenous immunoglobulin (IVIg) therapy for 5 days, her ataxic symptoms improved. Laboratory data were negative for antiganglioside antibody against GQ1b in the IgG subclass. Six months after her first admission, cognitive impairment gradually developed. She was re-admitted owing to new onset of unsteadiness while walking 1.5 years after her first admission. Diffusion-weighted brain MRI (DWI) revealed linear high-intensity signals in the region of the corticomedullary junction. Cutaneous skin biopsy revealed intranuclear inclusion bodies in sweat gland cells. Considering her family history along with the examination results, we diagnosed with adult-onset sporadic neuronal intranuclear inclusion disease (NIID). Retrospective investigation of the previous DWI obtained at the first admission had also shown slight linear high-intensity areas, suggesting that a series of events, including repeated sudden-onset transient ataxia, resulted due to NIID.

We describe a twenty-year follow-up study of antiglycolipid antibodies and electrophysiological results in a 36-year-old man with Campylobacter jejuni-associated Guillain-Barré syndrome (GBS). The patient had a high titer of IgG antibodies to GM1 and GA1 20 years ago. Plasma exchange resulted in full recovery from a bedridden status to independent walking in three weeks, except for residual mild weakness of the bilateral extensor hallucis longus muscles and atrophy of the plantar muscles. Twenty years later, he is unable to run at full pace due to neurological sequelae, and IgG antibodies to GM1 and GA1 were still slightly positive. This case suggests that marked improvement in the acute phase does not necessarily guarantee a subsequent good quality of life (QOL). Optional treatment such as complement inhibitors in the acute phase may be required to achieve better QOL in subsets of patients with GBS.

Promnesia is a feeling of familiarity and foreknowledge and is a manifestation of simple partial seizures (focal aware seizures). It is similar to déjà vu and has been reported to be a rare symptom in patients with temporal lobe epilepsy. Here, we investigated the clinical characteristics in 4 patients with partial epilepsy presenting promnesia. Three out of 4 patients showed abnormal electroencephalography (EEG) and/or MRI findings in the temporal lobe. Furthermore, in 2 patients, promnesia was the only aura. It is important to actively obtain medical history of patients about promnesia because this is useful for identifying the epileptic focus. Further cases need to be analyzed to evaluate the sensitivity and specificity of promnesia for diagnosis and therapy of partial epilepsy.