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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282XOn-line version ISSN 1678-4227

Arq. Neuro-Psiquiatr. vol.73 no.9 São Paulo Sept. 2015 


Longitudinally extensive transverse myelopathy in a patient with CADASIL

Mielopatia transversa longitudinalmente extensa em um paciente com CADASIL

Wladimir Bocca Vieira de Rezende Pinto1 

Paulo Victor Sgobbi de Souza1 

Acary Souza Bulle Oliveira1 

1Universidade Federal de São Paulo, Divisão de Doenças Neuromusculares, Departamento de Neurologia e Neurocirurgia, Sao Paulo SP, Brazil.

A 52-year-old Brazilian man presented with a 3-year-history of progressive cognitive decline, seizures and tetraparesis with sphincter disturbances. Familial history was positive for stroke-like episodes. Examination disclosed spastic tetraparesis with posterior cord syndrome. Neuroimaging revealed a longitudinally extensive centromedullary hyperintensity in the spinal cord between C2-T1 levels and a diffuse leukoencephalopathy (Figure), highly suggestive of Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL).

Figure Spinal cord involvement in CADASIL. 

CADASIL is a common hereditary vasculopathy in adults characterized by chronic migraine, subcortical infarcts and cognitive and behavioral disturbances1,2. Spinal cord involvement is extremely rare, making this a differential diagnosis of demyelinating diseases2,3.


. Andre C. CADASIL: pathogenesis, clinical and radiological findings and treatment. Arq Neuropsiquiatr. 2010;68(2):287-99. doi:10.1590/S0004-282X2010000200026 [ Links ]

. Hinze S, Goonasekera M, Nannucci S, Quaqhebeur G, Briley D, Markus HS et al. Longitudinally extensive spinal cord infarction in CADASIL. Pract Neurol. 2015;15(1):60-2. doi:10.1136/practneurol-2014-000870 [ Links ]

. Bentley P, Wang T, Malik O, Nicholas R, Ban M, Sawcer S et al. CADASIL with cord involvement associated with a novel and atypical NOTCH3 mutation. J Neurol Neurosurg Psychiatry. 2011;82(8):855-60. doi:10.1136/jnnp.2010.223297 [ Links ]

Received: January 25, 2015; Revised: April 08, 2015; Accepted: April 28, 2015

Correspondence: Wladimir Bocca Vieira de Rezende Pinto; UNIFESP, Departamento de Neurologia e Neurocirurgia; Rua Estado de Israel, 899; 04022-002 São Paulo SP, Brasil; E-mail:

Conflict of interest: There is no conflict of interest.

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.