Central Nervous System Magnetic Resonance Imaging Findings in Myotonic Dystrophy

Glantz, Russell H.; Wright, Robert B.; Huckman, Michael S.; Garron, David C.; Siegel, Irwin M.

doi:10.1001/archneur.1988.00520250042017

Cited by 87 publications

(42 citation statements)

References 9 publications

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“…The skull computerized tomography and magnetic resonance images did not disclose characteristic alterations 24 . Beyond alterations from cortical origin, some authors have described cytoplasmic eosinophilic bodies inclusion within neurons of the thalamus [25][26][27][28][29] .…”

Section: Discussion Discussion Discussion Discussionmentioning

confidence: 80%

The Kohs' blocks test as an important instrument to investigate the visuo-spatial impairments in myotonic dystrophy: part I. Quantitative and qualitative analysis

Wigg

Duro²

1999

Arq. Neuro-Psiquiatr.

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-This study presents the performance of 39 cases of myotonic dystrophy on Kohs' blocks test (21 females and 18 males, age range from 9 to 70 years). On this test, the patients have to reproduce figures from models previously showed to them. Some of the patients had some kind of professional activity, while others had never exerted a professional occupation. The patients denoted considerable difficulty to perform the test. Some cases constructed entirely different figures in comparison to the presented drawings, translating visuo-spatial and constructional disabilities. The performance was insufficient in 71.4 % of the cases. These cases solved less than 50% of the test. The levels of analysis and synthesis were severely impaired. A total of 18 cases got less than 10 points, not reaching 20% of the test. The results showed the sensitivity of this test in detecting visuo-spatial impairment in myotonic dystrophy.KEY WORDS: myotonic dystrophy, psychological tests, Kohs' blocks.O teste dos cubos de Kohs como importante instrumento para investigar as alterações visuo-espaciais da distrofia miotônica: I. Análise quantitativa e qualitativa RESUMO -Foram avaliados 39 casos de distrofia miotônica a partir do desempenho no teste dos cubos de Kohs. Havia 21 casos femininos e 18 masculinos, idade variando de 9 a 70 anos e diferentes níveis educacionais (do primário ao superior). Alguns encontravam-se ativos profissionalmente, enquanto outros nunca exerceram uma ocupação profissional. Os pacientes denotaram considerável dificuldade na execução deste teste que é instrumento que se apresenta fundamentalmente por reprodução de figuras. Houve casos que construíram figuras com formas bem diferentes dos desenhos apresentados. O desempenho foi insuficiente em 71,4 % dos casos, os quais não conseguiram resolver 50% do teste. Muitas das reproduções fugiram ao padrão correto, traduzindo comprometimento visuo-espacial pela grave distorção em comparação com a figura modelo. Os níveis de análise e síntese estavam comprometidos de forma importante. Dentre os pacientes, 18 casos não alcançaram os 10 pontos, dos 133 possíveis, não realizando sequer 20% do teste. Os resultados comprovaram a sensibilidade deste teste em detectar comprometimento visuo-espacial na distrofia miotônica. PALAVRAS-CHAVES: distrofia miotônica, testes psicológicos, cubos de Kohs, alterações visuo-espaciais.

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Section: Discussion Discussion Discussion Discussionmentioning

confidence: 80%

The Kohs' blocks test as an important instrument to investigate the visuo-spatial impairments in myotonic dystrophy: part I. Quantitative and qualitative analysis

Wigg

Duro²

1999

Arq. Neuro-Psiquiatr.

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“…In addition to the central nervous system [1,12], peripheral nerves are also involved [4,6,9,10,16,21,24]. Electrophysiological studies revealed a sensorimotor axonal neuropathy [21].…”

Section: Discussionmentioning

confidence: 99%

Comparative morphometric evaluation of peripheral nerves and muscle fibers in myotonic dystrophy

Wang

Schröder

2000

Acta Neuropathol

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We compared peripheral nerve fibers and muscle fibers in myotonic dystrophy (MD) using a computer-assisted device for morphometry. In the 17 cases with MD studied, the sural nerves of 14 cases (82%) showed various degrees of reduction of the myelin sheath area (MSA) per endoneurial area. Of these, 8 cases (47%) presented with a mild reduction of the MSA, 5 cases (29.4%) with moderate reduction, and one case (6%) with severe reduction. The number of myelinated nerve fibers was not significantly reduced in MD when compared with control nerves, due to clusters of small regenerated nerve fibers. The mean diameter of the muscle fibers in 6 of the 17 cases was less than 40 microm. Of these 6 severely affected cases, 5 revealed a considerable reduction of the MSA. Other cases, which appeared to be normal in respect to the diameter of muscle fibers, showed various degrees of reduction of the MSA. Thus, there is usually, but not always a morphometric correlation of the severity of changes between peripheral nerves and muscle. The severity of the peripheral neuropathy appears to depend largely on the patient's age, the stage of the disorder, and the time of progression. Electron microscopic examination of sural nerves showed significant, though non-specific pathological changes.

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“…Chang et al [7]and Meola et al [13]reported normal MRI or nonspecific white matter abnormalities in 17 and 15 DM patients, respectively, while Abe et al [4]reported WMLs in 14 patients consecutively examined. Glantz et al [14]found periventricular WMLs in 43% of patients, while Bachmann et al [15]found mainly subcortical WMLs. Some authors [6, 16]described characteristic anterior temporal WMLs, which were considered a volume averaging effect with the surrounding subarachnoid spaces by others [7].…”

Section: Introductionmentioning

confidence: 99%

Dilated Virchow-Robin Spaces in Myotonic Dystrophy: Frequency, Extent and Significance

Costanzo

Salle²,

Santoro³

et al. 2001

Eur Neurol

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To study the frequency, extent and significance of dilated Virchow-Robin spaces (VRSs) in dystrophia myotonica (DM), we evaluated the cranial magnetic resonance imaging (MRI) of 41 patients with the adult form of DM and 41 healthy controls. Dilated VRSs and white matter lesions (WMLs) were rated according to semiquantitative methods. Convexity VRSs were more frequent (68 vs. 34%; p < 0.01) and severer (median scores: 4 vs. 0; p < 0.01) in DM patients than in controls, while lenticulostriate VRSs did not show significant differences in frequency (83 vs. 70%; p > 0.05) and severity (median scores: 4 vs. 3; p > 0.05). WMLs were more frequent (66 vs. 22%; p < 0.01) and severer (median scores: 5 vs. 0; p < 0.01) in patients. Disease duration was negatively correlated with convexity VRSs and positively with lobar (centrum semiovale) WML scores. Dilated convexity VRSs might be one of the initial findings in cranial MRI of DM, preceding the appearance of lobar WMLs.

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Central Nervous System Magnetic Resonance Imaging Findings in Myotonic Dystrophy

Cited by 87 publications

References 9 publications

The Kohs' blocks test as an important instrument to investigate the visuo-spatial impairments in myotonic dystrophy: part I. Quantitative and qualitative analysis

The Kohs' blocks test as an important instrument to investigate the visuo-spatial impairments in myotonic dystrophy: part I. Quantitative and qualitative analysis

Comparative morphometric evaluation of peripheral nerves and muscle fibers in myotonic dystrophy

Dilated Virchow-Robin Spaces in Myotonic Dystrophy: Frequency, Extent and Significance

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