Vestibular Impairment in Frontotemporal Dementia Syndrome.

Background: No studies to date have attempted to evaluate frontotemporal lobar degeneration from the perspective of the vestibular system.

Objective: The present study examined vestibular function in patients with frontotemporal dementia (FTD) clinical syndrome and evaluated whether vestibular disorders are involved in the clinical symptoms due to FTD.

Methods: Fourteen patients with FTD syndrome, as well as healthy elderly controls without dementia, were included in the present study.

Galactorrhea in a Patient With Aquaporin-4 Antibody-positive Neuromyelitis Optica Spectrum Disorder: A Case Report and Review of the Literature.

This is the first report of a case of galactorrhea in a patient with neuromyelitis optica spectrum disorder (NMOSD) diagnosed on the basis of antiaquaporin-4 antibody seropositivity. The hypothalamus is becoming known as an area highly expressing aquaporin-4 and frequently involved in intracranial lesions of patients with neuromyelitis optica (NMO). We reviewed cases of hypothalamic endocrinopathy among patients with NMO, NMOSD, and the Japanese opticospinal form of MS.

Ectoine alters subcellular localization of inclusions and reduces apoptotic cell death induced by the truncated Machado-Joseph disease gene product with an expanded polyglutamine stretch.

Protein misfolding is considered a key event in the pathogenesis of polyglutamine disease such as Machado-Joseph disease (MJD). Overexpression of chaperone proteins and the application of chemical chaperones are reported to suppress polyglutamine induced cytotoxicity in vitro and in vivo. The effects of compatible solutes, which are osmoprotectants in bacteria and possess the action in stabilizing proteins under stress, have not, to our knowledge, been studied.

Early vestibular dysfunction in Machado-Joseph disease detected by caloric test.

The selective vulnerability of distinct neuronal structures is a major feature of Machado-Joseph disease (MJD), also known as spinocerebellar ataxia 3 (SCA3). Vestibular dysfunction is known to be a symptom of MJD, but little is known about precisely when the vestibular system becomes impaired. Using a caloric test, we evaluated vestibular function in 2 MJD patients.

[A case of sensory perineuritis with Bowen disease].

A 60-year-old man was admitted because of subacutely progressive sensory disturbance characterized by fluctuating dysesthesia and light pain on the skin of his hands and feet. He exhibited mononeuritis multiplex, and a plaque with sloughing was observed on the left side of his back. Sural nerve biopsy revealed marked thickening of the perineurium with vascularization, and no necrotizing vasculitis.