This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.
Abstract
Objective: To investigate the progression of cognitive impairment and its behavioral aspects in patients with SPG4-linked autosomal dominant hereditary spastic paraplegia (SPG4-ADHSP).
Methods: Sixteen patients, 45 years or older, from five families with SPG4-ADHSP were prospectively assessed. Eleven of these, from three families, were followed and 10 had two cognitive examinations using the Cambridge Cognitive Assessment (CAMCOG) 2.9 years apart. The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE), the Neuropsychiatric Inventory (NPI), and the Nurses’ Observation Scale for Geriatric Patients (NOSGER) were completed by close relatives of the 11 patients at the second assessment. Eleven matched control subjects had CAMCOG examinations 3.1 years apart.
Results: The mean CAMCOG score at the initial assessment was lower for the 10 HSP patients (73.5/107) than for 10 control subjects (91.7/107, p = 0.005). After 2.9 years, the HSP patients experienced a fall in the mean CAMCOG by 9.1 points to 64.4/107 (p = 0.008). The mean CAMCOG score for the control subjects (90.8/107) fell by only 0.9 points after 3.1 years (p = 0.36). The CAMCOG scores of two HSP patients moved from the mild (60 to 80) into the moderate dementia category (35 to 59) and in three other patients into the mild dementia range from borderline normal scores (81 to 85). IQCODE scores were abnormal in 9/11 HSP patients and 1/11 controls (p = 0.001). Seven of the 11 HSP patients were considered as having dementia.
Conclusion: This study indicates an active progression of cognitive deterioration and dementia in older patients with SPG4-ADHSP.
- Received May 5, 2003.
- Accepted September 24, 2003.
AAN Members
We have changed the login procedure to improve access between AAN.com and the Neurology journals. If you are experiencing issues, please log out of AAN.com and clear history and cookies. (For instructions by browser, please click the instruction pages below). After clearing, choose preferred Journal and select login for AAN Members. You will be redirected to a login page where you can log in with your AAN ID number and password. When you are returned to the Journal, your name should appear at the top right of the page.
AAN Non-Member Subscribers
Purchase access
Letters: Rapid online correspondence
REQUIREMENTS
You must ensure that your Disclosures have been updated within the previous six months. Please go to our Submission Site to add or update your Disclosure information.
Your co-authors must send a completed Publishing Agreement Form to Neurology Staff (not necessary for the lead/corresponding author as the form below will suffice) before you upload your comment.
If you are responding to a comment that was written about an article you originally authored:
You (and co-authors) do not need to fill out forms or check disclosures as author forms are still valid
and apply to letter.
Submission specifications:
- Submissions must be < 200 words with < 5 references. Reference 1 must be the article on which you are commenting.
- Submissions should not have more than 5 authors. (Exception: original author replies can include all original authors of the article)
- Submit only on articles published within 6 months of issue date.
- Do not be redundant. Read any comments already posted on the article prior to submission.
- Submitted comments are subject to editing and editor review prior to posting.
You May Also be Interested in
Alert Me
Recommended articles
Early MoCA predicts long-term cognitive and functional outcome and mortality after stroke
The Informant Questionnaire on Cognitive Decline in the Elderly (IQCODE) as a screening tool for dementia for a predominantly illiterate chinese population
Poststroke dementia
Autosomal dominant spastic paraplegia