The site of the Media Sphera Publishers contains materials intended solely for healthcare professionals.
By closing this message, you confirm that you are a certified medical professional or a student of a medical educational institution.

Login
EN
+7 (495) 482-4118
+7 (495) 482-0604
+8 800 250-18-12
  • (toll-free number for subscriptions)
    Mon-Fri from 10 a.m. to 6 p.m.

  • © 1998-2025
+7 (495) 482-43-29
EN
All journals
Journal
Year
Year
Search result: 0

Ovchinnikov A.V.

Military medical unit, Golytsino, Moscow region, Russia

Shprakh V.V.

Irkutskiĭ gosudarstvennyĭ institut usovershenstvovaniia vracheĭ

Clinical presentations of neurological forms of hepatolenticular degeneration in Primorsky Krai

Authors:

Ovchinnikov A.V., Shprakh V.V.

More about the authors

Journal: S.S. Korsakov Journal of Neurology and Psychiatry. 2016;116(11): 12‑16

DOI: 10.17116/jnevro201611611112-16

Read: 1354 times

Download PDF (RU)
Contact the author
Table of contents

CLINICAL PRESENTATIONS OF NEUROLOGICAL FORMS OF HEPATOLENTICULAR DEGENERATION IN PRIMORSKY KRAI
CLINICAL PRESENTATIONS OF NEUROLOGICAL FORMS OF HEPATOLENTICULAR DEGENERATION IN PRIMORSKY KRAI

To cite this article:

Ovchinnikov AV, Shprakh VV. Clinical presentations of neurological forms of hepatolenticular degeneration in Primorsky Krai. S.S. Korsakov Journal of Neurology and Psychiatry. 2016;116(11):12‑16. (In Russ.)
https://doi.org/10.17116/jnevro201611611112-16

Подписка 2026 Журнал неврологии и психиатрии им. С.С. Корсакова (S.S. Korsakov Journal of Neurology and Psychiatry)
 
МСФ на ЯМ
Использование инфографики авторами научных работ
Close metadata
Recommended articles:
Difficulties in life­time diagnosis of Creutzfeldt—Jakob disease. S.S. Korsakov Journal of Neurology and Psychiatry. 2024;(11):19-27
The use of arti­ficial inte­lligence in modern dentistry in the Russian Fede­ration. Stomatology. 2024;(5):42-45
Unre­solved problem: Chemotherapy-Induced Peri­pheral Neuropathy. P.A. Herzen Journal of Onco­logy. 2024;(5):76-81
Quality asse­ssment of health care deli­very to patients with sexually transmitted infe­ctions in dermatovenereological institutions of Moscow acco­rding to medi­cal reco­rds analysis. Russian Journal of Clinical Dermatology and Vene­reology. 2024;(5):503-509
Clinical case of auri­cular gouty tophi. Russian Bulletin of Otorhinolaryngology. 2024;(6):80-84
Contribution of Sergey Seli­vanovich Golo­vin to the deve­lopment of ophthalmology. Russian Annals of Ophthalmology. 2024;(6):159-163
Dete­ction of mpox virus by enzyme immu­noassay in a no-instrumental format and after its inactivation with formaldehyde. Mole­cular Gene­tics, Microbiology and Viro­logy. 2024;(4):49-53
Labo­ratory diagnostics of monkeypox: basic methods and prospects for new deve­lopments. Labo­ratory Service. 2024;(4):57-64
Obstructive sleep apnea syndrome as a predictor of atrial fibrillation. Russian Journal of Preventive Medi­cine. 2025;(1):109-114
Resu­lts of the All-Russian consensus conference on complications in bariatric surgery. Endo­scopic Surgery. 2025;(1):15-24
Abstract:

Clinical characteristics of neurological forms of hepatolenticular degeneration (HLD) were studied in one of the regions of Russia (Primorsky Krai) in the sample of 111 patients at the wide age range (average 35.6 years); mean time since the diagnosis was 13.7 years. According to N.V. Konovalov’s classification, 4 groups of patients were identified: I arrhythmic-hyperkinetic form (n=61), II extra-pyramidal cortical form (n=30), III tremulous form (n=15), IV tremulous-rigid form (n=6). A main body of clinical manifestations of the pathology at the stage of cerebral signal of HLD manifestation was determined; three degrees of the severity of its course were established. Correlation between clinical HLD manifestation severityand the time since the diagnosis of neurological stage of the disease and its form was noted. Age parameters of the development of different extra-pyramidal disturbances and predictors of the severity of the course were specified.

Keywords:

hepatolenticular degeneration
diagnosis
clinical presentations

Authors:

Ovchinnikov A.V.

Military medical unit, Golytsino, Moscow region, Russia

Shprakh V.V.

Irkutskiĭ gosudarstvennyĭ institut usovershenstvovaniia vracheĭ

Close metadata

References:

  1. Voloshin-Gaponov IK. Klinika cerebral'nyh narushenij pri gepatocerebral'noj degeneracii. Mezhdunarodnyj nevrologicheskij zhurnal. 2014;64(2):18-24. (In Russ.).
  2. Asanov AJu, Sokolov AA, Volgina SJa, Gorjacheva LG, Gustov AV, Ivanova-Smolenskaja IA. Kopishinskaja SV. i dr. Federal'nye klinicheskie rekomendacii po diagnostike i lecheniju bolezni Vil'sona—Konovalova (GLD). Proekt 05.12.13. M.: MZRF; 2013. (In Russ.).
  3. Ferenci P. Review article: diagnosis and current therapy of Wilson’s disease. Aliment Pharmacol Ther. 2004;19:157-165. doi: 10.1046/j.1365—2036.2003.01813.x
  4. Chetkina GS, Potapov AS, Cirjul'nikova OM. Bolezn' Vil'sona u detej: varianty manifestacii i trudnosti rannej diagnostiki. Voprosy diagnostiki v pediatrii. 2011;3(1):41-47. (In Russ.).
  5. Prashanth LK, Taly AB, Sinha S, Arunodaya GR, Swamy HS. Wilson’s disease: diagnostic errors and clinical implications. Journal Neurol Neurosurg Psychiatry. 2004;75:907-909. doi: 10.1136/Jnnp.2003.026310
  6. Moller LB, Horn N, Joppesen TD, Vissing J, Wibrand F, Jennum Р, Ott Р. Clinical presentation and mutations in Danish patients with Wilson Disease. European Journal Human Genetics. 2011;19:935-941. doi: 10.1038/ejhg.2011.80
  7. Ponomarev VV. Bolezn' Vil'sona—Konovalova «velikij hameleon». Mezhdunarodnyj nevrologicheskij zhurnal. 2010;3:161-165.(In Russ.).
  8. Lai C-H, Tseng HF. Population-based epidemiologic stude of Wilson’s disease in Taiwan. European Journal Neurology. 2010;17:830-833. doi: 10.111/j. 1468-1331.2009.02946
  9. Lee CC, Wu JY, Tai FJ. Molecular analysis of Wilson disease in Taiwan: Identification of one novel mutation and evidence of haplotype-mutation assotiation. Journal Hum Genet. 2000;45:275-279. doi: 10.1007/s100380070015
  10. Illarioshkin SN, Ivanova-Smolenskaja IA, Markova ED. DNK-diagnostika i mediko-geneticheskoe konsul'tirovanie v nevrologii. M.: Medicinskoe informacionnoe agentstvo. 2002. (In Russ.).
  11. Konovalov NV. Gepatocerebral'naja distrofija. M.: Medgiz; 1960. (In Russ.).
  12. Polishhuk VV, Fedotova EJu, Ivanova-Smolenskaja IA. Sluchaj gepatolentikuljarnoj degeneracii s debjutom nevrologicheskoj formy posle 45 let. Novosti mediciny i farmacii. 2013;458:39-42. (In Russ.).
  13. Taly AB, Prashanth LK, Sinha S. Wilson’s Disease: An Indian perspective. Neurology India. 2009;57:528-540. doi: 10.4103/0028-3886.57789
  14. Krysiak R, Handzlik-Orlik G, Okopien B. Endocrine symptoms as the initial manifestation of Wilson. The Yale Journal of Biology and Medicine. 2012;85:249-254.
Contact the author
Email
Message
The publishing house "Media Sphere" does not provide treatment services, does not give recommendations on contacting medical institutions and specific specialists, on the prescription of medicines and dietary supplements.

Email Confirmation

An email was sent to test@gmail.com with a confirmation link. Follow the link from the letter to complete the registration on the site.

Email Confirmation

Contact us
If you have any questions, complaints or suggestions, please use this feedback form.
Email
Message
The publishing house "Media Sphere" does not provide treatment services, does not give recommendations on contacting medical institutions and specific specialists, on the prescription of medicines and dietary supplements.
Submit Application

You can become an author of one of our magazines, send a request and we will consider it in during the day.

Name
Phone
E-mail
Message
Login
Registration
E-mail
Password
Forgot Password?

Log in to the site using your account in one of the services

Password recovery
E-mail
Login
Register

We use cооkies to improve the performance of the site. By staying on our site, you agree to the terms of use of cооkies. To view our Privacy and Cookie Policy, please. click here.