Dementia and Geriatric Cognitive Disorders
Original Research Article
Dementia with Impaired Temporal Glucose Metabolism in Late-Onset Metachromatic LeukodystrophyJohannsen P.a,b · Ehlers L.a · Hansen H.J.aaDepartment of Neurology and bPET Center, Aarhus University Hospital, Aarhus, Denmark
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Dement Geriatr Cogn Disord 2001;12:85–88
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Article / Publication Details
First-Page Preview
Published online: February 09, 2001
Issue release date: March – April
Number of Print Pages: 4
Number of Figures: 2
Number of Tables: 0
ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)
For additional information: https://www.karger.com/DEM
Abstract
An unusual case of very-late-onset metachromatic leukodystrophy (MLD) with dementia was studied. The patient was a 41-year-old male who presented with mild dementia and a single generalized tonic clonic seizure. Neuropsychological assessment demonstrated mild amnesia, visuospatial dysfunction and attention deficits with a slow psychomotor speed. MR brain imaging displayed confluent hyperintensities of periventricular and subcortical white matter. Low levels of arylsulfatase A confirmed the diagnosis. Impaired cortical glucose metabolism especially of the medial temporal and frontal cortices was observed using positron emission tomography and fluor-18-labeled fluorodesoxyglucose. The neuropsychological deficits are related to the location of deficits in glucose metabolism.
© 2001 S. Karger AG, Basel
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References
-
Alzheimer A: Beiträge zur Kenntnis der pathologischen Neuroglia und ihrer Beziehungen zu den Abbauvorgängen im Nervengewebe; in Nissl F, Alzheimer A (eds): Histologische und histopathologische Arbeiten über die Grosshirnrinde. Jena, Fischer, 1910, vol 3, pp 401–552.
-
Austin JH: Metachromatic form of diffuse cerebral sclerosis: I. Diagnosis during life by urine sediment examination. Neurology 1957;7:415–426.
- Muller D, Pilz H, Meulen VT: Studies on adult metachromatic leukodystrophy. 1. Clinical, morphological and histochemical observations in two cases. J Neurol Sci 1969;9:567–584.
- Mahon HH, Stone RK, Johnson R, Shah S: Biochemical abnormalities of metachromatic leukodystrophy in an adult psychiatric population. Am J Psychiatry 1981;138:1372–1374.
- Hyde TM, Ziegler JC, Weinberger DR: Psychiatric disturbances in metachromatic leukodystrophy. Insights into the neurobiology of psychosis. Arch Neurol 1992;49:401–406.
- Shapiro EG, Lockman LA, Knopman D, Krivit W: Characteristics of the dementia in late-onset metachromatic leukodystrophy. Neurology 1994;44:662–665.
- Hageman AT, Gabreels FJ, de Jong JG, Gabreels Festen AA, van den Berg CJ, van Oost BA, Wevers RA: Clinical symptoms of adult metachromatic leukodystrophy and arylsulfatase A pseudodeficiency. Arch Neurol 1995;52:408–413.
- Dousset V, Tison F, Vital A, Henry P: Leucodystrophie orthochromatique d’évolution rapide chez l’adulte. Rev Neurol (Paris) 1998;154:415–418.
- Wulff CH, Trojaborg W: Adult metachromatic leukodystrophy: Neurophysiologic findings. Neurology 1985;35:1776–1778.
- Fressinaud C, Vallat JM, Masson M, Jauberteau MO, Baumann N, Hugon J: Adult-onset metachromatic leukodystrophy presenting as isolated peripheral neuropathy. Neurology 1992;42:1396–1398.
-
World Health Organization: The ICD-10 classification of mental and behavioural disorders. Diagnostic criteria for research, ed 10. Geneva, WHO, 1993.
-
Exner JE: Rorschach interpretation assistance program. Version 3 (RISP3). Odessa, Psychological Assessment Resources, 1995.
-
Duyff RF, Weinstein HC: Late-presenting metachromatic leukodystrophy. Lancet 1996;348:1382–1383.
- Bosch EP, Hart MN: Late adult-onset metachromatic leukodystrophy. Dementia and polyneuropathy in a 63-year-old man. Arch Neurol 1978;35:475–477.
- Pilz H, Duensing I, Heipertz R, Seidel D, Lowitzsch K, Hopf HC, Goebel HH: Adult metachromatic leukodystrophy. I. Clinical manifestation in a female aged 44 years, previously diagnosed in the preclinical state. Eur Neurol 1977;15:301–307.
- Argyrakis A, Pilz H, Goebel HH, Muller D: Ultrastructural findings of peripheral nerve in a preclinical case of adult metachromatic leukodystrophy. J Neuropathol Exp Neurol 1977;36:693–711.
- Shapiro EG, Lipton ME, Krivit W: White matter dysfunction and its neuropsychological correlates: A longitudinal study of a case of metachromatic leukodystrophy treated with bone marrow transplant. J Clin Exp Neuropsychol 1992;14:610–624.
- Sadovnick AD, Tuokko H, Applegarth DA, Toone JR, Hadjistavropoulos T, Beattie BL: The differential diagnosis of adult onset metachromatic leukodystrophy and early onset familial Alzheimer disease in an Alzheimer clinic population. Can J Neurol Sci 1993;20:312–318.
- Skomer C, Stears J, Austin J: Metachromatic leukodystrophy (MLD). XV. Adult MLD with focal lesions by computed tomography. Arch Neurol 1983;40:354–355.
- Schipper HI, Seidel D: Computed tomography in late-onset metachromatic leucodystrophy. Neuroradiology 1984;26:39–44.
- Finelli PF: Metachromatic leukodystrophy manifesting as a schizophrenic disorder: Computed tomographic correlation. Ann Neurol 1985;18:94–95.
- Reider GI, Bornstein N: CT and MRI in late-onset metachromatic leukodystrophy. Acta Neurol Scand 1987;75:64–69.
- Ishii K, Kitagaki H, Kono M, Mori E: Decreased medial temporal oxygen metabolism in Alzheimer’s disease shown by PET. J Nucl Med 1996;37:1159–1165.
Article / Publication Details
First-Page Preview
Published online: February 09, 2001
Issue release date: March – April
Number of Print Pages: 4
Number of Figures: 2
Number of Tables: 0
ISSN: 1420-8008 (Print)
eISSN: 1421-9824 (Online)
For additional information: https://www.karger.com/DEM
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