Sensory disturbances in Creutzfeldt-Jakob disease

被引:1
作者
Appel, Shmuel [1 ,2 ]
Cohen, Yael [2 ]
Appel, Shira [3 ]
Cohen, Oren S. [3 ,4 ]
Chapman, Joab [3 ,5 ,6 ]
Rosenmann, Hanna [7 ]
Nitsan, Zeev [1 ,2 ]
Kahana, Esther [1 ,2 ]
机构
[1] Barzilai Univ, Dept Neurol, Med Ctr, Ashqelon, Israel
[2] Ben Gurion Univ Negev, Fac Hlth Sci, Beer Sheva, Israel
[3] Tel Aviv Univ, Sackler Fac Med, Tel Aviv, Israel
[4] Assaf Harofeh Med Ctr, Dept Neurol, Zerifin, Israel
[5] Sagol Neurosci Ctr, Dept Neurol, Ramat Gan, Israel
[6] Chaim Sheba Med Ctr, Ramat Gan, Israel
[7] Hadassah Hebrew Univ, Agnes Ginges Ctr Human Neurogenet, Dept Neurol, Med Ctr, Jerusalem, Israel
关键词
CJD; PNS; E200K; Sporadic; Sensory; PERIPHERAL NERVOUS-SYSTEM; PRION PROTEIN;
D O I
10.1007/s10072-023-07093-0
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
BackgroundCreutzfeldt-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.MethodsThe 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.ResultsEighty-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).ConclusionsSensory symptoms are more common in E200K patients and often follow peripheral neuropathy distribution that suggests PNS involvement.
引用
收藏
页码:1057 / 1062
页数:6
相关论文
共 22 条
  • [11] MUTATION OF THE PRION PROTEIN IN LIBYAN JEWS WITH CREUTZFELDT-JAKOB DISEASE
    HSIAO, K
    MEINER, Z
    KAHANA, E
    CASS, C
    KAHANA, I
    AVRAHAMI, D
    SCARLATO, G
    ABRAMSKY, O
    PRUSINER, SB
    GABIZON, R
    [J]. NEW ENGLAND JOURNAL OF MEDICINE, 1991, 324 (16) : 1091 - 1097
  • [12] Prion diseases
    Knight, RSG
    Will, RG
    [J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY, 2004, 75 : I36 - I42
  • [13] Kobayashi Atsushi, 2018, Handb Clin Neurol, V153, P207, DOI 10.1016/B978-0-444-63945-5.00012-X
  • [14] Accumulation of prion protein in the peripheral nervous system in human prion diseases
    Lee, CCM
    Kuo, LT
    Wang, CH
    Scaravilli, F
    An, SF
    [J]. JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 2005, 64 (08) : 716 - 721
  • [15] Sensory features of variant Creutzfeldt-Jakob disease
    Macleod, MA
    Stewart, GE
    Zeidler, M
    Will, R
    Knight, R
    [J]. JOURNAL OF NEUROLOGY, 2002, 249 (06) : 706 - 711
  • [16] Genetic Factors in Mammalian Prion Diseases
    Mead, Simon
    Lloyd, Sarah
    Collinge, John
    [J]. ANNUAL REVIEW OF GENETICS, VOL 53, 2019, 53 : 117 - 147
  • [17] Impairment of the peripheral nervous system in Creutzfeldt-Jakob disease
    Niewiadomska, M
    Kulczycki, J
    Wochnik-Dyjas, D
    Szpak, GM
    Rakowicz, M
    Lojkowska, W
    Niedzielska, K
    Inglot, E
    Wieclawska, M
    Glazowski, C
    Tarnowska-Dziduszko, E
    [J]. ARCHIVES OF NEUROLOGY, 2002, 59 (09) : 1430 - 1436
  • [18] Redefining periodic patterns on electroencephalograms of patients with sporadic Creutzfeldt-Jakob disease
    Shin, Jung-Won
    Yim, Byeongsoo
    Oh, Seung Hun
    Kim, Nam Keun
    Lee, Sang Kun
    Kim, Ok-Joon
    [J]. CLINICAL NEUROPHYSIOLOGY, 2017, 128 (05) : 756 - 762
  • [19] Sorgato M Catia, 2006, Ital J Biochem, V55, P222
  • [20] Epidemiological and clinical characteristics of patients with late-onset Creutzfeldt-Jakob disease
    Trachtenbroit, Iliya
    Cohen, Oren S.
    Chapman, Joab
    Rosenmann, Hanna
    Nitsan, Zeev
    Kahana, Esther
    Appel, Shmuel
    [J]. NEUROLOGICAL SCIENCES, 2022, 43 (07) : 4275 - 4279