Inflammatory myopathies are a heterogeneous group of immune-mediated diseases that involve skeletal muscle as well as many other organs. The classification of inflammatory myopathies has been based on clinical diagnoses, pathological diagnoses, and autoantibodies, independently. Antisynthetase syndrome, characterized by myositis, interstitial lung disease, skin rash, arthropathy, and Raynaud phenomenon, is a clinical entity based on the presence of aminoacyl transfer RNA synthetase (ARS) antibodies in patients’ serum. A cohort study of muscle biopsy entitled “Integrated Diagnosis Project for Inflammatory Myopathies” revealed that of 460 patients with idiopathic inflammatory myopathies, 51 (11%; female:male, 31:20) had antisynthetase myopathy. It is noted that anti-OJ antibodies, one of anti-ARS antibody subtypes, are clearly detected by RNA immunoprecipitation, but not conventional detection methods including line blot and enzyme-linked immunosorbent assays. The combined mean onset age of the patients was 60 years (range 13–85 years). There were no significant HLA-DRB1 alleles associated with anti-ARS antibodies. All patients with antisynthetase myopathy patients presented muscle limb weakness; 14 had severe weakness, 17 neck weakness, 15 dysphagia, and 15 muscle atrophy. Although patients with anti-OJ antibodies showed severe muscle weakness, the clinical presentations defined by anti-ARS antibodies were relatively homogeneous. In muscle pathology, perifascicular necrosis is a distinctive hallmark of antisynthetase myopathy. Patients with antisynthetase myopathy responded to the combination of immunosuppressive therapy, with favorable outcomes. However, interstitial lung disease, found in 41 patients, was more closely related to mortality than myositis. Antisynthetase myopathy has a distinct clinical and histological entity among idiopathic inflammatory myopathies.
Recently, in the field of stroke rehabilitation, some novel concepts and therapeutic interventions have been proposed. It seems that earlier mobilization for acute stroke patients could lead to better functional outcome. In addition, neural plasticity during acute phase of stroke is enhanced, which means that this phase of stroke could be the period when the patients are likely to respond to rehabilitation training. In the future, acute rehabilitation should be aggressively provided in stroke centers in Japan. Some interventions such as non-invasive brain stimulation, centrally-acting drugs and vagus nerve stimulation have been reported to enhance neural plasticity. If these interventions are introduced combined with rehabilitation training, compensatory mechanism for impaired neurological function could be facilitated, leading to further functional recovery. Some robotic devices to support joint movements of the limbs externally have been developed. Robot-assisted rehabilitation can improve the efficacy of rehabilitation training, especially when applied for gait training. Neurofeedback is a sophisticated training system applying real-time monitoring of brain activity with the use of functional neuroimaging. Neurofeedback can be introduced in order to remedy motor imagery of stroke patients even if motor function is severely impaired. Regenerative therapy is a promising therapeutic intervention and some institutions in Japan have already started to introduce this therapy for stroke patients. It is proposed that rehabilitation training should be provided following the introduction of regenerative therapy so that structural reorganization caused by the therapy could lead to beneficial functional reorganization of the damaged brain. With the aim of improving active motor functions of hemiparetic limbs, botulinum toxin injection for limb spasticity after stroke should be combined with rehabilitation training. If these concepts and interventions are introduced aggressively and more widely for stroke patients, it is expected that functional outcome of such patients could be generally improved.
In amyloid β-related angiitis, cortical or subcortical microbleeding or cortical superficial siderosis supports clinical diagnosis. However, here we present a 75-year-old female case of amyloid β-related angiitis that did not initially show these lesions. The patient developed right homonymous hemianopia and aphasia, and subsequently became comatose. Her brain lesions progressed extensively from the left occipital lobe to the bilateral cerebral hemispheres, with diffused leptomeningeal lesions and scattered DWI high-intensity lesions. After pathological diagnosis, steroid treatment improved her symptoms as well as imaging findings. No hemorrhagic lesions were detected in the T2*-weighted imaging performed before treatment. However, susceptibility-weighted imaging performed after treatment showed a number of lesions with microbleeding. The clinical features of amyloid β-related angiitis that do not show hemorrhagic lesions at onset should be investigated for rapid therapeutic intervention in the future.
Three patients with neurodegenerative diseases who had developed repeated aspiration pneumonia underwent laryngeal closure, a surgical procedure at the larynx to prevent aspiration. None of these patients have developed aspiration pneumonia since the procedure. One patient needed endoscopic suction and cough assist machine to clear thick sputum, because tracheostomy bypassed the upper airway and so prevented moisturization of inhaled air. While two patients achieved freedom from tracheal cannulation, one needed continued cannulation because of narrowing of the stoma due to improvements in the nutritional condition. One patient was able to resume oral intake. Although the right timing to perform the procedure and optimal care along with long-term observation are important, laryngeal closure is an effective option for patients with neurodegenerative diseases to prevent recurrent aspiration pneumonia.
A 51-year-old man with a past history of hypothyroidism suddenly became comatose after a few days of general malaise and headache. On admission to our hospital, his consciousness level was Japan Coma Scale III-200, but no focal neurological deficits were evident. Serum anti-thyroglobulin antibody was >4,000 IU/ml and anti-thyroid peroxidase antibody was 265 IU/ml. Well characterized neuronal antibodies were not fully examined in this case, but based on high titers of serum thyroid antibodies, methylprednisolone pulse therapy was started under diagnosis of suspected Hashimoto encephalopathy. Although methylprednisolone pulse therapy was effective, every time the dose of oral prednisolone was reduced, relapse attacks similar to the first episode occurred, a total of seven times. At each relapse, cerebrospinal fluid findings and MRI findings were normal. Plasmapheresis and azathioprine were added, until two years after onset, when further acute neurological attacks no longer occurred, but attention and memory impairments persisted. Relapse in Hashimoto encephalopathy is not rare; careful, long-term follow-up is needed.
Central nervous system intravascular lymphoma sometimes includes multiple lesions mimicking cerebral infarction. Herein, we report our experience with a case of intravascular large B-cell lymphoma (IVLBCL) with multiple hemorrhages. A 53-year-old woman was admitted to our hospital with clonic convulsions of the left upper limb. Brain MRI revealed a large number of high-intensity areas on FLAIR and low-intensity areas on susceptibility-weighted imaging (SWI). Chest CT showed bilateral multiple high-density lesions in the lungs. Biopsy of pulmonary lesions revealed no abnormal cells. Levetiracetam was administered to prevent the seizures that were assumed to occur due to the cerebral cortex lesions; however, convulsive seizure recurred with a depressed level of consciousness. On a repeat brain MRI examination, severe, multiple new lesions were shown to have developed bilaterally in the cerebral cortex and white matter, exhibiting spotty low intensities on SWI. Biopsy of a new cerebral lesion was carried out and the lesion was pathologically diagnosed as IVLBCL with hemorrhages. IVLBCL should be noted as one of the differential diagnoses not only in case with multiple infarct lesions, but also in case with multiple hemorrhages.
A 62-year-old woman was transported to our hospital for management of generalized clonic seizures. Cerebrospinal fluid examination showed an increased monocyte-dominant cell count, high protein concentration, and low glucose concentration that was 17% of the plasma glucose concentration. Contrast-enhanced cranial magnetic resonance imaging revealed diffuse leptomeningeal enhancement with multiple nodular lesions. She underwent examinations that ruled out the following conditions: tuberculous meningitis, systemic sarcoidosis, malignant lymphoma, carcinomatous meningitis, and central nervous system vasculitis. On hospital day 13, dural and brain biopsies revealed neurosarcoidosis, for which steroid therapy was administered. Thereafter, imaging examinations showed marked improvement. Because isolated neurosarcoidosis is difficult to diagnose, early pathologic diagnosis may be essential.
A 33-year-old male was admitted to our hospital due to bilateral optic neuritis (ON) and transverse myelitis (TM), which occurred almost simultaneously. Spinal MRI showed the longitudinally extensive TM, located from C2 to conus. Serum anti-aquaporin 4 antibody was negative. He was tentatively diagnosed as seronegative neuromyelitis optica spectrum disorders (NMOSD). During the clinical course, serum anti-myelin oligodendrocyte glycoprotein (MOG) antibody was detected, and finally he was diagnosed as anti-MOG antibody positive neurologic disease (MOG-ND). Our case highlighted that early detection of MOG antibody should be considered in male cases with clinical manifestation of classical Devic’s disease, such as simultaneous disease onset of bilateral ON or ON + TM.
We report three female patients, aged 77, 89, and 92 years, with arterial emboli in their limbs that developed before and after recombinant tissue plasminogen activator (rt-PA) treatment for cerebral infarction. Arterial embolism in one limb developed in two patients before rt-PA treatment and in one during rt-PA treatment at the time of the first medical examination. Thrombectomy was performed in two patients. In all patients, the arterial emboli of the extremities were accompanied by acute cardiogenic cerebral emboli. Patients with cardiogenic cerebral emboli can also develop emboli in the extremities. Particularly, during rt-PA treatment of cerebral infarction, the presence of other possible thromboembolisms, in addition to hemorrhagic complications and changes in neurological symptoms, should be examined.