Hemiplegic migraine is a rare subtype of migraine characterized by reversible hemiplegia/hemiparesis as an aura symptom. Visual, sensory, and/or speech symptoms typically accompany the motor aura, followed by throbbing headache. Because motor symptoms may last for up to several weeks, this unique clinical entity can markedly impair quality of life. Familial hemiplegic migraine is caused by autosomal dominant mutations in the CACNA1A, ATP1A2, SCN1A, and PRRT2 genes. These mutations are thought to induce neuronal hyperexcitability with resultant enhanced synaptic transmission and increased susceptibility to cortical spreading depolarization/depression. Therapeutic options remain limited, owing to the contraindication of triptans for acute treatment and the lack of established preventive therapies. However, recent evidence supports the potential efficacy of calcitonin gene–related peptide–targeting monoclonal antibodies.

The Japanese Society of Neurology’s Committee on Measures for Transition from Pediatric to Adult Health Care held a workshop to discuss the activities of the transitional care initiatives undertaken by various healthcare professionals. The following points were addressed: (1) The Osaka Transitional Care Support Center reported on assistance for patients and families through consultations and pre-transition conferences; (2) The Shikoku Medical Center for Children and Adults introduced a collaborative approach in which transitional support is developed together with children and their families; (3) A staff member with dual qualifications as a certified genetic counselor and a certified intractable disease nurse described transitional care practices at Kanazawa University Hospital; and (4) The Tokyo Metropolitan Transitional Care Support Center presented several programs promoting transitional care, including a “Support Program for Guardians of Patients with Decision-making Difficulties” and a “Chronic Disease Transition App.” Collectively, these presentations highlighted the importance of advancing transitional care through effective multidisciplinary collaboration.

Beta-propeller protein-associated neurodegeneration (BPAN) is a neurodegeneration with brain iron accumulation (NBIA) disorder caused by autophagy abnormalities. Clinical features of BPAN include global developmental delay in early childhood, followed by progression of cognitive dysfunction and parkinsonism in adulthood. A 32-year-old woman diagnosed with BPAN and confirmed by genetic analysis showed motor symptoms that rapidly progressed after the age of 30 years. Baclofen was administered for spasticity, though a high fever and elevated serum CK level were observed, and the symptoms persisted for several months even after stopping the drug. It was suspected that rhabdomyolysis have been due to muscle tissue fragility associated with autophagy impairment in BPAN, in combination with increased muscle tone.

A 68-year-old female was admitted to our hospital because of a right-sided headache since one year, an inability to perform housework, and a decrease in speech since March of that year. Upon admission, higher brain dysfunctions, including attention disorder, aphasia, and apraxia, were observed. Contrast-enhanced MRI revealed dural thickening in the bilateral frontal to parietal lobes and FLAIR imaging revealed high signal intensities in the bilateral frontal brain sulcus. Blood tests revealed a high IgG4 level (273 mg/dl). Dural biopsy revealed infiltration of IgG4-positive plasma cells into the tissue, and IgG4-related hypertrophic pachymeningitis was diagnosed. Administration of prednisolone resulted in resolution of the dural thickening, an absence of FLAIR imaging high signal intensities of the cerebral sulcus, and a considerable improvement of the higher brain dysfunctions. In patients with headache and higher brain dysfunctions, hypertrophic pachymeningitis should be considered in the differential diagnosis.

Fisher syndrome (FS) is characterized by acute extraocular muscle paralysis, ataxia, and absent tendon reflexes, along with positive anti-GQ1b antibodies. However, incomplete forms of the syndrome, in which only some of the symptoms are present, are known to exist. In this report, we present two cases of incomplete Fisher syndrome with unilateral ocular motor dysfunction. Both patients were positive for anti-GQ1b antibodies and exhibited mild ataxia as the only other neurological symptom, but improved without treatment intervention. This case report highlights the need to recognize that incomplete forms of this syndrome, presenting with unilateral ocular muscle paralysis, exist within the disease spectrum.

A 75-year-old woman presented with dysarthria and sensory deficit of the left upper and lower limbs. Brain MRI findings revealed a cerebral infarction in the right middle cerebral artery territory. One year prior to admission, she underwent a Maze procedure and surgical left atrial appendage closure to treat her atrial fibrillation, which restored her sinus rhythm. Consequently, she discontinued anticoagulant therapy two months before admission. However, transesophageal echocardiography revealed a left atrial thrombus, which was considered the cause of the cerebral infarction. Treatment with oral anticoagulants was initiated, and the left atrial thrombus resolved. There was no recurrence of cerebral infarction. Since left atrial thrombus formation can occur even after surgical treatment for atrial fibrillation, careful follow-up is required.

The patient was a 30-year-old man who was admitted to our department 2 months after the onset of anterograde amnesia. Cognitive function tests indicated memory impairment only. MRI FLAIR images of the head showed a high signal in the bilateral medial temporal lobes. Corticosteroid therapy did not improve his symptoms, and he subsequently developed status epilepticus. Concurrently, his cerebrospinal fluid was positive for anti-leucine-rich glioma-inactivated 1 (LGI1) antibody. He was diagnosed with anti-LGI1 antibody-positive encephalitis. Plasma exchange therapy and intravenous immunoglobulin therapy improved the status epilepticus, but memory impairment and bilateral medial temporal lobe atrophy remained. This case was characterized by memory impairment that only lasted for 2 months after onset and a poor response to corticosteroid therapy.

A 32-year-old previously healthy man developed abnormal behavior two days after the onset of fever. He had experienced epistaxis during consecutive work days prior to the onset. While clinical findings suggested encephalitis, blood cultures yielded methicillin-resistant Staphylococcus aureus (MRSA). No obvious local infectious foci were identified in the skin or soft tissues; however, brain MRI revealed multiple embolic-like lesions. The MRSA strain produced markedly elevated toxic shock syndrome toxin-1 (TSST-1), suggesting a toxic shock syndrome–like state. Although initial empirical therapy did not cover MRSA, targeted anti-MRSA agents were started following its identification. Assuming an occult infectious focus, intravenous anti-MRSA therapy was continued for 6 weeks after blood cultures converted to negative. He was discharged on hospital day 54 without neurological sequelae. Community-associated MRSA can cause severe sepsis even in young individuals, and encephalopathy may be the initial presentation. Clinicians should consider MRSA sepsis in the differential diagnosis of patients presenting with encephalitis-like symptoms. In this case, the preceding epistaxis suggested that nasal colonization served as the portal of entry.