Viswanathan et al. present a thoughtful, timely, and alternative approach to the classification of dementia. [1] Specifically, they stress the importance of vascular factors on Alzheimer disease (AD) and of viewing dementia as a continuum of purely neurodegenerative dementia, purely vascular dementia, and as the combination of both vascular and degenerative contributions seen in some cases.
The consideration of other etiologies is also important in the evaluation of dementia. Other diseases may clinically present like AD, including hereditary diffuse leukoencephalopathy with spheroids (HDLS).
HDLS is an autosomal dominant condition with progressive cognitive and motor dysfunction and sporadic patients (sporadic DLS) have been reported. Lack of awareness of this disease may lead to misdiagnosis. In our report of an American family with HDLS, the proband's father-with pathology characteristic of HDLS-was clinically diagnosed with vascular dementia. [2] The report by Viswanathan et al [1] also discusses the importance of neuroimaging in the workup of dementia. Although heterogeneous, MRI changes in HDLS are frequently progressive and confluent white matter hyperintensities with frontal prominence and associated atrophy.
Familiarity with the MR appearance of HDLS is significant, even if MR findings do not always allow definite diagnosis. Histopathology confirmation is also needed.
References
1. Viswanathan A, Rocca WA, Tzourio C. Vascular risk factors and dementia: how to move forward? Neurology 2009;72:368-374.
2. Van Gerpen JA, Wider C, Broderick DF, Dickson DW, Brown LA, Wszolek ZK. Insights into the dynamics of hereditary diffuse
leukoencephalopathy with axonal spheroids. Neurology 2008;71:925-929.
Editor’s Note: The authors of the article were offered the opportunity to respond but declined.
Disclosure: Dr. Wszolek serves as Co-Editor-in-Chief for Parkinsonism and Related Disorders; Co-Editor-in-Chief for the Polish Edition of Neurology; Regional Editor of European Journal of Neurology; and Editorial Board Member for Neurologia Neurochirurgia Polska; Advances in Rehabilitation; Medical Journal of the Rzeszow University; and Clinical and Experimental Medical Letters.
Dr. Wszolek has not yet accrued revenue from the following filed patents: Norwegian Patent Office, Patent # NO20045612A0, Polynukleotid; Norwegian Patent Office, Patent # NO20052535A0, Polynucleotide; InventorsNorwegian Patent Office, Patent # NO0323175B1; Framgangsmate for a pavise en mutasjon som forarsaker arvelig parkinsonisme; Australian Patent Office, Patent # AU5319787AA; A novel polynucleotide involved in heritable Parkinson’s disease; World Patent Office, Patent # WO06068492A1; A NOVEL POLYNUCLEOTIDE INVOLVED IN HERITABLE PARKINSON’S DISEASE; USA Patent Office, Patent # US2008/0009454 A1; Polynucleotide; Canadian Patent Office, Patent # CA2606672AA; A NOVEL POLYNUCLEOTIDE INVOLVED IN HERITABLE PARKINSON’S DISEASE; Canadian Patent Office, Patent # CA2606672AA; A NOVEL POLYNUCLEOTIDE INVOLVED IN HERITABLE PARKINSON’S DISEASE; European Patent Office, Patent # EP1838871A1; A NOVEL POLYNUCLEOTIDE INVOLVED IN HERITABLE PARKINSON’S DISEASE.
Dr. Broderick and Dr. Schweitzer report no disclosures.