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.

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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.

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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.

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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.

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The patient was a 43-year-old woman. She had been aware of progressive muscle weakness since around the age of 30. Two months ago, she began to have difficulty making sense of conversation. She was transported to our hospital after choking, and upon arrival, she was found to have generalized muscle weakness and myotonia, along with disorientation. A brain MRI revealed widespread high-intensity lesions in the cerebral white matter on FLAIR images, findings similar to gliomatosis cerebri (GC). Genetical analysis revealed a diagnosis of dystrophia myotonica type 1 (DM1). A brain biopsy revealed a diffuse astrocytoma. There have been no previous reports of DM1 associated with diffuse astrocytoma and imaging findings similar to GC, and we report this patient together with a literature review.

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Androgenetic alopecia (AGA) is commonly treated with finasteride and minoxidil, which are both recommended as grade A therapies in the Japanese Dermatological Association guidelines. However, their association with thromboembolic events and ischemic stroke remains unclear. We report a 40-year-old man without apparent risk factors for stroke who developed acute ischemic stroke due to left internal carotid artery occlusion while taking finasteride (1 ‍mg/‍day) and minoxidil (10 ‍mg/‍day). Mechanical thrombectomy retrieved a dark red thrombus primarily composed of red blood cells and fibrin. This case represents a rare report of internal carotid artery occlusion with pathologically confirmed thrombus in a patient receiving these medications. Finasteride may promote hypercoagulability via androgen suppression–induced estrogen predominance, and minoxidil may contribute through blood pressure–lowering effects. Intravenous alteplase and thrombectomy were effective treatments, and discontinuation of the medications is recommended for secondary prevention.

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Here, we report a man clinically diagnosed with behavioral variant frontotemporal dementia triggered by a long-standing walking habit, and pathologically diagnosed with corticobasal degeneration at death (aged 78 years). Walking habits began approximately 65 years of age for diabetes management, involving daily walks of 7–8 ‍km along the same route. Unlike stereotypical roaming, his persistent walking with a transfixed expression suggested an impulse-control disorder in which the urge to walk could not be suppressed, combined with compulsive, ritualistic behavior. Lesion distribution in corticobasal degeneration involved the bilateral frontal lobes, anterior temporal lobes, and basal ganglia, predominantly in the frontal convexity. Functional decline in these areas may contribute to prolonged walking habit.

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We report two cases of unilateral lesion of the inferior colliculus presenting with distinctive tinnitus and hearing impairment. Case 1 was a 40-year-old woman under treatment for multiple sclerosis who developed sudden tinnitus in her bilateral ears, initially resembling a “propeller”-like sound, which evolved into high-pitched “hyun-hyun” tones and subsequently into sustained “hyuun-hyuun” tones. She also exhibited impaired perception of spoken language. Brain MRI revealed a new small lesion in the right inferior colliculus. Case 2 was a 49-year-old man who experienced sudden onset of tinnitus characterized by alternating cicada-like, bell-like, and electronic “pyuun-pyuun” sounds. MRI revealed a small hemorrhage in the right inferior colliculus. In both cases, the characteristics of tinnitus changed over time and was accompanied by impaired recognition of speech sounds. These findings suggest that unilateral inferior colliculus lesions, a relay nucleus of the auditory pathway, can cause temporally evolving tinnitus and selective hearing deficits, symptoms that have been rarely reported.

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Herein, we present the case of a 67-year-old woman who presented with a 14-month history of progressive lower limb paresthesia and muscle weakness. Bilateral sensorineural hearing loss also developed during the disease course. Six months prior to admission, cerebrospinal fluid cytology revealed findings suspect of malignancy. Although tumor screening was performed, the cause could not be identified. Due to symptom progression, the patient was re-admitted after which further examination revealed invasive lobular carcinoma of the left breast as the primary tumor, with further metastasis to the large intestine also confirmed. Immunostaining of the cerebrospinal fluid revealed cells with positive findings for estrogen receptor (ER) and AE1/3, leading to a definitive diagnosis of meningeal dissemination. This was a rare case of meningeal dissemination of breast cancer, initially presenting with slowly progressive cauda equina syndrome. This case highlights the importance of repeated tumor screening for neurological symptoms of unknown primary origin.

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The patient was a 36-year-old woman with normal birth and development. The patient experienced gradual memory loss from the age of 29 years old. The patient showed irritability at 35, convulsion at 36, and was diagnosed to have early-onset dementia. Family history of early-onset dementia was suspected in her mother and her brother. Brain MRI showed atrophy mainly in parietal lobe and posterior cingulate cortes. Amyloid PET was positive and cerebrospinal fluid showed decreased amyloid-beta 42/40 ratio and increased phospho-tau 181, leading to a diagnosis of Alzheimer’s disease (AD). Genomic analysis revealed known pathogenic variant c.617G>A (p.G206D) in PSEN1. The clinical presentation of very-early-onset memory loss followed by psychiatric symptoms and convulsion matched previous reports of the same variant. This patient represents the first reported case in Japan carrying the p.G206D variant and has the youngest age at onset among previously reported cases. This case highlights the importance of considering AD even in very-early-onset cases and detailed family history taking is important in earlier diagnosis.

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A 77-year-old man was treated for Nocardia abscessus brain abscess with high-dose trimethoprim-sulfamethoxazole (TMP-SMX) and ceftriaxone (CTRX). During the treatment, he developed consciousness disturbance and negative myoclonus (NM) in all four limbs. Initially, we suspected CTRX-induced neurotoxicity because the clinical phenotype, characterized by NM, strongly resembled cephalosporin encephalopathy. However, we considered CTRX unlikely to be the cause because the patient’s renal function was preserved, and NM appeared during the period when CTRX was withheld. Consequently, we suspected TMP-SMX as the causative agent, despite such symptoms being rare for this drug. Discontinuation of TMP-SMX alone, while continuing CTRX, resulted in the rapid disappearance of involuntary movements and improvement in consciousness. This clinical course confirmed the diagnosis of TMP-SMX-induced neurotoxicity. Although TMP-SMX encephalopathy typically presents with psychosis, high-dose administration can cause NM mimicking cephalosporin neurotoxicity. In cases under treatment with high-dose of TMP-SMX and preserved renal function, clinicians should consider that TMP-SMX can cause NM.

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The patient was a 34-year-old man who sustained multiple injuries in a traffic accident, including multiple rib fractures, splenic injury, and a right temporal bone fracture. To evaluate the splenic injury, contrast-enhanced CT scans were performed multiple times; however, the patient developed nausea and was subsequently diagnosed with iodine hypersensitivity. Five years after the traffic accident, he began experiencing diplopia, prompting an MRI scan that revealed a cerebral infarction in the right midbrain. As he continued to suffer recurrent cerebral infarctions, cerebral angiography using gadolinium-based contrast agents was performed, which identified a dissecting aneurysm in the left vertebral artery. Neurological symptoms may appear in a delayed fashion following traffic trauma, and even in patients with iodine hypersensitivity, gadolinium-based contrast agents can aid in diagnosis.

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This article released online on July 11, 2015 as advance publication was retracted by author’s request.

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This article released online on July 11, 2015 as advance publication was retracted by author’s request.

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