Sensory disturbances in Creutzfeldt-Jakob disease

Shmuel Appel, Yael Cohen, Shira Appel, Oren S. Cohen, Joab Chapman, Hanna Rosenmann, Zeev Nitsan, Esther Kahana

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disease characterized by rapidly progressive dementia, motor impairments, and psychiatric symptoms. Sensory disturbances were occasionally reported as well. The study aims to describe the sensory symptoms of the disease. Methods: The CJD Israeli National Database was screened for patients who presented sensory symptoms throughout the disease course. Symptoms, characteristics, and distribution were reviewed and the demographic and clinical data (sex, etiologies of the disease, age of onset, disease duration, neurological exam finding, tau protein level, EEG and MRI findings) were compared with the demographics and clinical data of CJD without sensory symptoms. Then, the patients with sensory symptoms were divided into patients with symptom distribution consistent with peripheral nervous system (PNS) involvement and central nervous system (CNS) involvement. The demographics and clinical data of the 2 groups were compared. Results: Eighty-four CJD patients with sensory symptoms and 645 CJD patients without sensory symptoms were included in the study. Sensory symptoms were more common in genetic E200K CJD patients (14.6% vs. 5.6% respectively, p = 0.0005) (chi-squared test). Numbness and neuropathic pain were the most common symptoms and distribution of symptoms of “stocking gloves” with decreased deep tendon reflexes suggesting peripheral neuropathy in 44% of the patients. In these patients, the classical EEG findings of Periodic Sharp Wave Complexes were less often found (58% vs. 22%, p = 0.02) (chi-squared test). Conclusions: Sensory symptoms are more common in E200K patients and often follow peripheral neuropathy distribution that suggests PNS involvement.

Original languageEnglish
Pages (from-to)1057-1062
Number of pages6
JournalNeurological Sciences
Volume45
Issue number3
DOIs
StatePublished - 1 Mar 2024

Keywords

  • CJD
  • E200K
  • PNS
  • Sensory
  • Sporadic

ASJC Scopus subject areas

  • Clinical Neurology
  • Psychiatry and Mental health
  • Dermatology

Fingerprint

Dive into the research topics of 'Sensory disturbances in Creutzfeldt-Jakob disease'. Together they form a unique fingerprint.

Cite this