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The Differential Diagnosis of Adult Onset Metachromatic Leukodystrophy and Early Onset Familial Alzheimer Disease in an Alzheimer Clinic Population

Published online by Cambridge University Press:  18 September 2015

A.D. Sadovnick ,
H. Tuokko ,
D.A. Applegarth ,
J.R. Toone ,
T. Haijistavropoulos  and
B.L. Beattie
A.D. Sadovnick*
Affiliation:
Departments of Medical Genetics (A.D.S.), Psychiatry (H.T.), Paediatrics (D.A.A., J.R.T.) and Psychology (T.H.), University of British Columbia and Clinic for Alzheimer Disease and Related Disorders, University Hospital-UBC Site (A.D.S., H.T., T.H., B.L.B.), Vancouver Received February 3, 1993. Accepted in final form July 22, 1993
H. Tuokko*
Affiliation:
Departments of Medical Genetics (A.D.S.), Psychiatry (H.T.), Paediatrics (D.A.A., J.R.T.) and Psychology (T.H.), University of British Columbia and Clinic for Alzheimer Disease and Related Disorders, University Hospital-UBC Site (A.D.S., H.T., T.H., B.L.B.), Vancouver Received February 3, 1993. Accepted in final form July 22, 1993
D.A. Applegarth*
Affiliation:
Departments of Medical Genetics (A.D.S.), Psychiatry (H.T.), Paediatrics (D.A.A., J.R.T.) and Psychology (T.H.), University of British Columbia and Clinic for Alzheimer Disease and Related Disorders, University Hospital-UBC Site (A.D.S., H.T., T.H., B.L.B.), Vancouver Received February 3, 1993. Accepted in final form July 22, 1993
J.R. Toone*
Affiliation:
Departments of Medical Genetics (A.D.S.), Psychiatry (H.T.), Paediatrics (D.A.A., J.R.T.) and Psychology (T.H.), University of British Columbia and Clinic for Alzheimer Disease and Related Disorders, University Hospital-UBC Site (A.D.S., H.T., T.H., B.L.B.), Vancouver Received February 3, 1993. Accepted in final form July 22, 1993
*
Department of Medical Genetics, University of British Columbia, #226-2174 University Boulevard, Vancouver, British Columbia V6T 1Z3
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Abstract:

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Clinical differentiation between forms of progressive dementia can prove difficult, particularly when relatively rare forms of dementia are involved. Factors such as family history of dementia, age at onset, presenting features such as personality change, cognitive deficits, psychiatric symptoms, and clinical course (progressive deterioration; retention of skills over time) may prove useful for directing investigations to identify underlying pathology and genetic implications. This is illustrated by two patient reports. Each patient had the onset of memory/behavioral problems at approximately age 40 years, was initially given a psychiatric, non-dementing diagnosis, and had a positive family history for early onset behavioral and memory problems. After longitudinal assessment, the diagnosis of Alzheimer disease was confirmed at autopsy in one patient and a diagnosis of familial, adult-onset metachromatic leukodystrophy in the other.

Type
Research Article
Information
Canadian Journal of Neurological Sciences , Volume 20 , Issue 4 , November 1993 , pp. 312 - 318
Copyright
Copyright © Canadian Neurological Sciences Federation 1993

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