Identification of small fiber neuropathy in neuronal intranuclear inclusion disease: A clinicopathological study.

ABSTRACT:

Introduction

Neuronal intranuclear inclusion disease (NIID) manifests as dementia combined with other neurological symptoms. However, small fiber neuropathy (SFN) and pathology remain unknown in NIID.

Methods

A total of 294 subjects, including patients with NIID, Parkinson's disease, Alzheimer's disease, diabetic peripheral neuropathy, and healthy controls (HCs), were included. Clinical scales, sensory and autonomic function testing, and skin biopsy were performed.

Results

NIID patients had more severe sensory and autonomic dysfunction than other groups. Substantial reductions in intraepidermal, sweat gland, and pilomotor nerve fiber densities were observed in NIID patients, with a non-length dependent pattern. Detailed analysis revealed marked reductions in noradrene

SUBMITTER: Liu M 

PROVIDER: S-EPMC11847649 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature