Internal medicine
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Huntington's disease (HD) is a dominantly inherited neurological disorder characterized by chorea, psychiatric symptoms, and cognitive decline but typically lacks muscular atrophy and weakness. We herein report a case of genetically confirmed HD showing progressive systemic weakness with findings of upper and lower motor neuron involvement due to amyotrophic lateral sclerosis (ALS). The current patient and the previously reported cases with complications of HD and ALS indicate that cytosine-adenine-guanine (CAG) repeat expansion in the huntingtin gene might have a pathogenic role in causing the two neurological disorders.
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An 86-year-old woman was admitted to our hospital with cryptogenic progressive dyspnea and dysphagia following a tracheostomy procedure 4 months prior to presentation. She exhibited fluctuating diplopia, bilateral vocal fold paralysis, normal nerve test results, negative findings for serum anti-acetylcholine receptor and anti-muscle-specific kinase antibodies, and positive findings for anti-LDL receptor-related protein 4 (LRP4). ⋯ Consequently, the patient was diagnosed with double-seronegative myasthenia gravis (DSN-MG) and began immunomodulatory therapy. This case emphasizes the diagnostic challenges of bulbar-type DSN-MG and underscores the value of a VFSS with edrophonium for diagnosing this condition.
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Case Reports
Acute Hemorrhagic Leukoencephalitis with Concurrent Retinal Vasculitis in an Elderly Japanese Patient: A Case Report.
A 68-year-old Japanese man developed a fever, headache, hiccups, and altered consciousness. Brain magnetic resonance imaging revealed a hemorrhagic lesion in the right temporal lobe and multiple high-intensity white matter lesions. ⋯ Corticosteroids enabled full recovery. Although AHLE is uncommon in elderly individuals, clinicians should be aware of its occurrence in this patient subgroup and recognize potential retinal manifestations associated with AHLE.
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A 79-year-old woman developed herpes zoster ophthalmicus (HZO) with a vesicular rash on the nasal root, which developed soon after intravenous acyclovir therapy. Although varicella zoster virus DNA was undetectable in the cerebrospinal fluid, she presented with ophthalmoplegia without optic nerve dysfunction 32 days after the onset of HZO. ⋯ Ophthalmoplegia did not immediately respond to these therapies but resolved 4 months later. We should be aware that ophthalmoplegia can occur, even after HZO and meningitis are completely treated.
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A 52-year-old Japanese man with a history of childhood asthma presented at our emergency department with progressive dyspnea. Despite subcutaneous adrenaline injections, salbutamol nebulization, and intravenous methylprednisolone, the carbon dioxide partial pressure (pCO2) increased to 110 mmHg. The patient was intubated, and mechanical ventilation was initiated because of severe respiratory failure. ⋯ Therefore, we decided to administer biologics. After the administration of a single dose of benralizumab, his respiratory condition improved, with normalization of pCO2, tidal volume, and airway resistance. We successfully extubated the patient two days after the administration of benralizumab.