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We herein report the distribution of gray matter lesions on magnetic resonance imaging (MRI) in two patients with lymphomatosis cerebri (LC). In our patients, the fluid-attenuated inversion recovery sequence of brain MRI demonstrated a bilateral and diffuse high signal intensity, not only in the white matter but also in the thalamus, globus pallidus, putamen, and hippocampus. Among the deep gray matter, the caudate head and putamen (striatum) were relatively spared when compared with the globus pallidus, thalamus, and hippocampus. Interestingly, we found seven previous reports of similar MRI findings, with relative sparing of the striatum, in patients with LC. This finding may be characteristic of LC and help facilitate its diagnosis. Further investigations of a larger number of LC patients are necessary to confirm these findings.

Introduction: Brachiocephalic artery stenosis rarely causes right hemispheric infarction with associated left hemiparesis. To date, there have been no reported cases of stroke associated with brachiocephalic artery stenosis that were successfully treated with recombinant tissue-type plasminogen activator (rt-PA), alteplase. Case Report: An 80-year-old woman presented with left hemiparesis. Brain computed tomography showed no hemorrhage, and computed tomography angiography demonstrated brachiocephalic artery stenosis. Alteplase was administered based on a diagnosis of ischemic stroke. Brain magnetic resonance imaging showed multiple acute infarctions. Thereafter, the blood pressure of the right arm was found to be lower than that of the left arm. The patient’s neurological deficits gradually improved; she was eventually able to walk again and was thus discharged home. Conclusion: While the combination of left hemiparesis and a decrease in blood pressure in the right arm are well known in patients with stroke associated with Stanford type A aortic dissections, it may also occur in patients with stroke due to brachiocephalic artery stenosis. Unlike stroke associated with Stanford type A aortic dissections, stroke due to brachiocephalic artery stenosis may be treated with alteplase.

Encephalopathy is a rare side effect of cephalosporin treatment. We herein present a case of encephalopathy induced by ceftriaxone, a third-generation cephalosporin, in a patient with renal failure. An 86-year-old woman on maintenance hemodialysis received ceftriaxone for Helicobacter cinaedi bacteremia. Her mental status deteriorated during antibiotic treatment, and an electroencephalogram revealed triphasic waves predominantly in the frontal area. Her consciousness improved after the discontinuation of the antibiotic due to the suspicion of ceftriaxone-induced encephalopathy. This is the first reported case of encephalopathy associated with high plasma and cerebrospinal fluid ceftriaxone concentrations, and provides significant evidence for a causal relationship between the administration of ceftriaxone and the onset of encephalopathy.

Myasthenia gravis (MG), a neuromuscular junction autoimmune disease, sometimes complicates second malignancies; however, T-cell lymphoproliferative disorders have rarely been reported. A 55-year-old man, who received oral tacrolimus and prednisolone for MG for 16 years after thymectomy, presented with left abdominal pain, lymphadenopathy, and splenomegaly. A lymph node biopsy revealed peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). This is the first report of oral tacrolimus leading to a T-cell lymphoproliferative disorder in patient without a history of transplantation. Physicians should be aware of the possibility of rare T-cell lymphoproliferative disorders, such as PTCL-NOS, occurring as complications in MG patients on immunosuppressive regimens after thymectomy.

Based on previous reports, we propose a practical guide to choose dabigatran 150 mg twice daily or apixaban 5 mg twice daily for patients with atrial fibrillation. We recommend the use of dabigatran 150 mg twice daily for patients with atrial fibrillation who have a high risk of embolism (e.g., ischemic stroke on other oral anticoagulants, presence of left atrial appendage thrombus) and a low risk of bleeding. However, the prevalence of such patients with atrial fibrillation is considered low because patients with atrial fibrillation with a high risk of embolism usually have a high risk of bleeding. In most other patients with atrial fibrillation, the use of apixaban 5 mg twice daily should be considered.

Background Depression is a common symptom after stroke, but its neural substrates remain unclear. The ascending serotonergic system originates from the raphe nuclei in the brainstem. We hypothesized that depressive disorder due to brainstem infarction is associated with damage to the raphe nuclei. Methods We prospectively enrolled 19 patients who had the first-ever acute isolated brainstem infarction in an observational cross-sectional study. All patients were evaluated by using the Montgomery Asberg Depression Rating Scale (MADRS), the clinician-rated version of Apathy Evaluation Scale (AES-C) and Mini-Mental State Examination (MMSE). Depressive disorder was diagnosed according to DSM-5 and MADRS score of 12 or greater. Diffusion tensor imaging and proton density-weighted images were used to identify damage in the raphe nuclei. Accordingly, patients were classified into either the raphe-nuclei–damaged or intact group. Prevalence of depressive disorder and the MADRS, AES-C, and MMSE scores were compared between the two groups. Results Depressive disorder was more frequent in the damaged group (n=6) than in the intact group (n=13) (83% vs. 15%; P=0.01). MADRS scores were higher in the damaged group than in the intact group (mean±1 SD, 17.5±7.9 vs. 7.0±4.4; P=0.002), whereas the AES-C and MMSE scores did not differ between groups. Limitations We did not assess the damage to the ascending projection fibers from the raphe nuclei. Conclusions Our results suggest that damage to the raphe nuclei underlies depressive disorder due to brainstem infarction, possibly via serotonergic denervation.

Background Oral function deteriorates easily during the acute phase of cerebral stroke. Therefore, oral health care involving a transdisciplinary approach consisting of dental and medical professionals might be important, but has not been studied in detail. Objective This study assessed the oral health status of patients with cerebral stroke in the acute phase, with the aim of elucidating the efficacy of collaborative, transdisciplinary oral health care involving dentists, dental hygienists, nurses and speech therapists. Methods The participants were 115 consecutive acute cerebral stroke patients, who received oral health care while hospitalised at the university hospital. Their oral health status was assessed using the oral health assessment tool (OHAT) on admission and discharge. Results Patients with acute cerebral stroke had high OHAT scores on admission, meaning poor oral health status. The collaborative oral health care resulted in significant decrease of OHAT scores at discharge, indicative of the improvement of oral health status. Multivariate analysis identified OHAT score for tongue, dentures and oral cleanliness on admission as the significant variables associated with poor oral health status at discharge. Conclusions Thus, the oral health of cerebral stroke patients in the acute phase can be improved by implementing transdisciplinary collaboration of medical and dental professionals. Particularly, patients with problems pertaining to the tongue, dentures and oral cleanliness as revealed through OHAT on admission may require more intensive intervention.

A 58-year-old man consulted our hospital due to a 2-year history of dysarthria and a 1-month history of blepharospasm. In addition to the ataxic dysarthria and blepharospasm, a neurological examination demonstrated slight ataxia of the trunk and lower limbs. Brain MRI demonstrated atrophy of the upper portion of the cerebellar vermis. Gene analysis established a diagnosis of spinocerebellar ataxia type 31 (SCA31). Single photon emission computed tomography (SPECT) with the three-dimensional stereotaxic ROI template (3DSRT) software program demonstrated hyperperfusion in the lenticular nucleus and thalamus. Although the association between SCA31 and blepharospasm in our patient remains unclear, we considered that this combination might be more than coincidental.

Left hemispatial neglect (neglect) is an impaired state of spatial attention. We aimed to reconstruct structural connectivity in the spatial attention network and to identify disconnection patterns underlying neglect. We enrolled 59 right-handed patients who had their first-ever infarction in the right hemisphere and classified them into neglect group (34 patients with neglect) and control group (25 patients without neglect). The neglect group was further subcategorized into 6 subgroups based on infarcted vascular territories. Diffusion tensor imaging data were obtained from all patients. Fractional anisotropy maps were compared between neglect group/subgroups and the control group by using non-parametric voxel-based analysis, generating a lesion path mask. Probabilistic tractography analysis using the lesion path mask reconstructed the following structural connectivity in the spatial attention network, which is specifically damaged in neglect patients: (1) superior longitudinal fasciculus (SLF) I connecting the superior parietal lobule/intraparietal sulcus with the superior frontal gyrus/frontal eye field (SFG/FEF) (dorsal attention network); (2) SLF III/the arcuate fasciculus (AF) and the extreme capsule/inferior fronto-occipital fasciculus (IFOF) connecting the right inferior parietal lobule/temporoparietal junction/superior temporal gyrus (IPL/TPJ/STG) with the middle frontal gyrus/inferior frontal gyrus (ventral attention network); (3) the thalamic radiations to the spatial attention-related cortices; and (4) SLF II and IFOF interconnecting dorsal and ventral attention networks. Individual analysis indicated that isolated damage in SLF I, SLF II, SLF III/AF or the thalamic radiations to IPL/TPJ/STG due to posterior cerebral artery infarction, or simultaneous damage in four thalamic radiations due to anterior choroidal artery infarction, underlies different phenotypes of neglect.

A 74-year-old right-handed woman without cognitive impairment suddenly developed nonfluent aphasia. Brain MRI showed acute infarction in the right frontal lobe and insula without involvement of the corpus callosum. A neurological examination demonstrated not only transcortical motor aphasia, but also ideomotor apraxia and right hand predominant constructional apraxia (CA). To date, right hand predominant CA has only been reported in patients with corpus callosum lesions. The right hand predominant CA observed in our patient may be associated with the failure to transfer information on the spatial structure from the right hemisphere to the motor cortex of the left hemisphere.

Although progressive multifocal leukoencephalopathy (PML) cases showing responses to mefloquine therapy have been reported, the efficacy of mefloquine for PML remains unclear. We report on the failure of mefloquine therapy in two Japanese patients with PML unrelated to human immunodeficiency virus. One of the patients was a 47-year-old male who had been treated with chemotherapy for Waldenstrom macroglobulinemia, and the other was an 81-year-old male with idiopathic CD4 + lymphocytopenia. Diagnosis of PML was established based on MRI findings and increased JC virus DNA in the cerebrospinal fluid in both patients. Mefloquine was initiated about 5months and 2months after the onset of PML, respectively. During mefloquine therapy, clinical and radiological progression was observed, and JC virus DNA in the cerebrospinal fluid was increased in both patients. Both patients died about 4months and 2months after initiation of mefloquine, respectively. Further studies are necessary to clarify the differences between mefloquine responders and non-responders in PML.

A 79-year-old man presented with a slowly progressive gait disturbance. Brain MRI demonstrated ventriculomegaly and the hummingbird sign. A lumbar puncture showed no abnormalities of the cerebrospinal fluid. The improvement of the gait disturbance after the ventriculoperitoneal shunt led to a diagnosis of idiopathic normal pressure hydrocephalus. Interestingly, postoperative brain MRI demonstrated the disappearance of not only ventriculomegaly, but also the hummingbird sign. The disappearance of the hummingbird sign suggests that an increase in the cerebrospinal fluid in the lateral and third ventricles could cause the compression of the superior surface of the midbrain tegmentum, which manifests as the hummingbird sign.

In this study we aim to establish a motor nerve conduction study (NCS) for the cauda equina and examine its usefulness in multifocal motor neuropathy (MMN) and amyotrophic lateral sclerosis (ALS). NCS of the tibial nerve proximal to the knee was performed with an optimized high-voltage electrical stimulation (HV-ES) method in 21 normal subjects, 5 with MMN, and 11 with ALS. HV-ES, but not magnetic stimulation, could supramaximally stimulate the cauda equina. Cauda equina motor conduction time determined by HV-ES, but not that with F-waves, correlated well with cauda equina length on magnetic resonance imaging. HV-ES revealed proximal lesions in 4 MMN patients but in none of the ALS patients. Importantly, 1 patient with "MMN without conduction block (CB)" had a CB in the cauda equina. Cauda equina motor conduction is better evaluated by HV-ES than with F-wave study or magnetic stimulation. HV-ES can help to distinguish MMN and "MMN without CB" from ALS.

Background: Nasu–Hakola disease (NHD) is a rare autosomal recessive disorder, characterized by a combination of progressive presenile dementia and formation of multifocal bone cysts, caused by genetic mutations of DAP12 and TREM2, which constitute a receptor/adapter signaling complex expressed on osteoclasts, dendritic cells, macrophages, and microglia. No Japanese patients with TREM2 mutations have been reported previously. Methods: We reported three siblings affected with NHD in a Japanese family. Amongst them, two died of NHD during the fourth decade of life. The analysis of genomic DNA, cDNA cloning, and western blot of lymphocyte proteins was performed on samples of the living patient. The transcriptome was studied in the autopsied brain of one patient. Results: We identified a homozygous conversion of a single nucleotide T to C at the second position of intron 3 in the splice-donor consensus site (c.482+2T>C) of the TREM2 gene, resulting in exon 3 skipping and aberrant expression of truncated proteins. We identified 136 upregulated genes involved in inflammatory response and immune cell trafficking and 188 downregulated genes including a battery of GABA receptor subunits and synaptic proteins in the patient’s brain. Conclusions: This is the first report of a Japanese NHD family caused by a splicing mutation of TREM2 that induces both neuroinflammation and neurodegeneration.

Hydrophilic fluorescent derivatization reagents, sensors and probes are of great value because of their use in accurate analyses under water-rich condition. In this study, 13 7-substituted-2,1,3-benzoxadiazole-4-sulfonates (called SBD derivatives) as representatives of ionic benzofurazans were synthesized and their absorption and fluorescence spectra were obtained in solvents of varying polarity. The fluorescence quantum yields ( Φ f ) of the investigated compounds were significantly affected by the substituent at the 4-position. Solvent effects on the Φ f values were also observed. It was found that the combination of the PM3 and PM3-CAS/CI (CI = 6) methods afforded a good relationship between the experimental and calculated S 1 ← S 0 transition energies of the SBD derivatives.

A 42-year-old man presented with fever, headache and liver dysfunction, and was diagnosed as having aseptic meningitis by lumbar puncture. The PCR detected Epstein-Barr virus (EBV)-DNA in the peripheral blood and cerebrospinal fluid. About 20 days after onset, the patient presented with hearing impairment in the right ear, which was confirmed by a pure tone audiogram. The hearing acuity improved after the initiation of hydrocortisone sodium phosphate. We presume that the hearing impairment was due to auditory nerve neuritis related to extension of inflammation of the meninges. This is the first reported case of EBV-associated meningitis showing hearing impairment.

A 25-year-old man suffered from recurrent, mass-like, intracerebral lesions and was diagnosed with tumefactive multiple sclerosis (MS). Both interferon β-1b and oral methotrexate (7.5 mg weekly) could not suppress frequent relapses, but he had been in remission for 8 months by the mitoxantrone treatment. At the age of 27, the patient developed a partial seizure. Brain MRI revealed a lesion in the right frontal lobe, and intensive investigation excluded etiologies other than MS. Thereafter, the patient complained of dysesthesia around the left shoulder. Neurological examination revealed sensory loss in left dermatomal regions from C3 to T1 levels (Picture 1). However, tendon reflexes were exaggerated in the left arm, and there was no weakness or long tract sign. Follow-up brain MRI showed a subcortical lesion in the right postcentral gyrus (arrowheads in Picture 2, A-C) consistent with the pseudoradicular sensory loss (1, 2), according to the sensory homunculus (3). The latencies of near-field potentials evoked by the median nerve stimulation (the potential at Erb’s point, N11, and N13) were within normal ranges, and cervical MRI revealed no lesion in the spinal cord and nerve roots. MS often attacks the spinal cord and causes sensory loss with a sensory level, but it rarely involves the parietal lobe to cause cortical sensory loss (4). Careful neurological examination led us to target the MRI precisely and make the correct anatomical diagnosis for our patient.