RESUMO
A 60 year old man with chronic lymphocytic leukemia (CLL) developed a subacute neurological illness associated with multiple contrast enhancing lesions on CT scan. At autopsy large demyelinating lesions characteristic of progressive multifocal leukoencephalopathy (PML) were found in the right cerebral hemisphere surrounded by a dense leukemic infiltrate. The areas of contrast enhancement, highly unusual for PML, coincided with the CLL infiltrate.
Assuntos
Neoplasias Encefálicas/patologia , Leucemia Linfoide/patologia , Leucoencefalopatia Multifocal Progressiva/patologia , Tomografia Computadorizada por Raios X , Lobo Frontal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Lobo Parietal/patologiaRESUMO
Involvement of the peripheral nervous system by amyloidosis is common. It is less well recognized that amyloid can directly infiltrate and weaken skeletal muscle. We report a case of a 73-year-old woman, known to have cardiac amyloidosis, who developed profound weakness secondary to amyloid myopathy. Review of the 8 other well documented cases in the literature has revealed a rather homogeneous syndrome. Proximal weakness, muscle stiffness, pseudohypertrophy and myalgia constitute the principal features. This syndrome usually develops in cases with well recognized generalized amyloidosis. Amyloid is deposited within the basal lamina of blood vessels and muscle fibers.