Telemedicine is not widely used in patients with intractable neurological disorders including neurodegenerative diseases even though its application was covered by national insurance in Japan. To clarify what patient factors should be overcome for telemedicine application, we surveyed outpatients with intractable neurological disorders using questionnaires in Tottori University Hospital, Tekeda General Hospital and Fukuoka University Hospitals. The survey showed that more than half of the telemedicine-beginners did not want to use telemedicine in the former two hospitals located in provincial cities (0.2 to 0.3 million population), but more than half would like to use it in the large hospital sited at one of the regional core cites (1.5 million population). Binomial logistic analysis of the beginners revealed that predictors for patients to choose “I do not want to use telemedicine” were “no experience using internet at home” and “need of a helper to use internet equipment for telemedicine at home”. The odds ratios of the other factors such as the concerns about the cost or quality of outpatient medical practices were low. On the other hand, most of already telemedicine users wanted to continue telemedicine. The present study revealed that the quality of internet connection environment is the most critical factor for patients to use telemedicine. It must be requisite for wide use of telemedicine to establish a good internet connection environment at areas/home and to supply good equipment to the patients for the connection.

A 72-year-old woman with limb weakness was admitted to our hospital. Her symptoms began just one month prior to presentation and showed gradual progression, leading to difficulties in physical movement. She had undergone breast cancer surgery at the age of 70 years. Neoadjuvant chemotherapy with epirubicin, cyclophosphamide, and docetaxel was administered, and the patient subsequently underwent a simple mastectomy and axillary lymphadenectomy. Neurological examination revealed bilateral relative afferent pupillary defects, severe muscle weakness in the limbs, hyporeflexia of the lower extremities, sensory disturbances below Th1 level, dysuria, and constipation. MRI revealed a left internal capsule lesion, extensive longitudinal transverse myelitis at C4–Th10 level, nerve root enlargement in the extraspinal canal, and high bilateral cervical/lumbar cord intensity. Nerve conduction studies revealed bilateral demyelinating polyneuropathy of the median nerves. Positive anti-AQP4 antibodies in both serum and cerebrospinal fluid ‍led to the diagnosis of neuromyelitis optica spectrum disorder (NMOSD). The patient’s symptoms improved after immunosuppressive treatment. Here, we describe the case of a patient with AQP4 autoantibody-positive NMOSD who demonstrated nerve root enlargement in the extraspinal canal and peripheral nervous system damage during demyelination.

A 32-year-old man presented with the symptoms of a floating sensation, weakness on the right side of the body, and tremor of the right hand. Head MRI was performed, and T₂-weighted images showed high-signal lesions around the lateral ventricles, subcortical white matter, and dorsal medulla oblongata. Moreover, MRI of the cervical spine showed multiple high-signal lesions without contrast enhancement. Based on these findings, the patient was diagnosed with primary progressive multiple sclerosis (PPMS) and was treated with steroid pulse therapy, plasma exchange, and oral fingolimod. However, the patient’s condition deteriorated slowly, and he died at the age of 43 years. An autopsy revealed multiple demyelinating lesions in the central nervous system. No inflammatory cell infiltration or macrophage accumulation was observed, and there was no evidence of an active lesion. Herein, we present this rare autopsy case of PPMS in Japan with a review of the literature.

A woman in her 60s had been experiencing numbness in her hands and feet for two weeks prior to the hospitalization, and had difficulty walking. Five years ago, eradication therapy for Helicobacter pylori was attempted twice, but was deemed unsuccessful. Current symptoms: redness of the tongue, stocking-glove type paresthesia, decreased deep sensation in the lower limbs, and trunk ataxia. Blood vitamin B12 levels were low, and a thoracic spinal cord MRI showed symmetric high signals in the posterior columns of the spinal cord. Subacute combined degeneration of the spinal cord was suspected, and vitamin B12 injections were started, allowing the patient to walk independently. Anti-intrinsic factor and anti-gastric parietal cell antibodies were positive, and atrophic gastritis was found on gastroscopy. Based on the above, it was determined that the cause was impaired vitamin B12 absorption due to autoimmune gastritis. In recent years, it has been pointed out that autoimmune gastritis is common among cases of repeated failure of H. pylori eradication, and there is a possibility that this may lead to vitamin B12 deficiency neurological disorders.

The patient was a male in his 30s, under treatment for Crohn’s disease of the small intestine since his 20s. He was admitted to the hospital due to worsening renal function. Two days after admission, he suddenly developed left upper and lower extremity paralysis followed by generalized convulsions and decreased level of consciousness. Head MRI showed multiple abnormal signal areas in the bilateral cerebral white matter and multiple microhemorrhages inside the lesions. CSF examination showed 11,136 cells/mm³ (98% polymorphonuclear cells, 2% mononuclear cells), 311 ‍mg/dl protein, 76 ‍mg/dl sugar, and MBP > 2,000 pg/ml. Chest X-ray showed infiltrate and multiple nodular shadows in both lung fields, and venous blood culture detected Staphylococcus epidermidis. Her general condition and neurological symptoms gradually improved after continuation of antimicrobial agents, methylprednisolone pulse therapy, and continuous hemodiafiltration. Acute hemorrhagic leukoencephalitis is a type of severe acute disseminated encephalomyelitis, and cases of marked improvement such as the present case are rare. We report this case with some discussion.

A 65-year-old man was admitted to our hospital following a sudden loss of consciousness and seizures. He presented with fever, and a head MRI revealed lesions in the left medial and lateral temporal lobes, as well as the thalamus. Initially, he was treated for suspected limbic encephalitis, including herpes simplex encephalitis (HSE). While his seizures resolved, aphasia persisted. Subsequent tests ruled out infections or autoimmune encephalitis, and a repeat MRI showed dilation of the left Labbe’s vein. Further vascular examination, including MRA, 4D-CTA, and digital subtraction angiography (DSA), confirmed the presence of a dural arteriovenous fistula (dAVF), which drained from the left occipital artery and middle meningeal artery via the left Labbe’s vein into the superior sagittal sinus. The patient underwent transarterial embolization for treatment. This case highlights the need to consider a dAVF in the differential diagnosis when a patient presents with seizures, fever, and temporal lobe lesions.

An 88-year-old woman with a history of two prior episodes of vertigo developed a mild leftward head tilt approximately four months after initiating pregabalin for severe tingling in the distal extremities. Two months later (day 1), she sustained a head injury without loss of consciousness. On day 22, she acutely developed leftward lateral flexion of the head while standing. Her gait progressively deteriorated, and she presented to our clinic on day 38. Neurological examination revealed mild disturbance of consciousness and marked leftward tilt of the head and trunk. Cranial MRI demonstrated bilateral chronic subdural hematomas without intra-axial lesions, including in the brainstem or cerebellum. Following surgical evacuation, the abnormal posture of the head and trunk resolved promptly. A horizontal, direction-fixed, right-beating nystagmus was observed, suggesting left-sided vestibular hypofunction. Subtle postural imbalance due to vestibular hypofunction and adverse effects of pregabalin may have been decompensated by the subdural hematomas, resulting in the pronounced cervical and truncal tilt.