Congophilic angiopathy with cerebrospinal symptoms

A patient is reported who developed the first symptoms of spinal motor neuron affection 20 years prior to his death at the age of 79. In the course of the disease dementia and spasticity of the legs occurred. The patient died of metastasizing carcinoma of the colon. The autopsy revealed amyloid angiopathy of the brain and cervical spinal cord, corresponding to the clinical symptomatology. So far, 11 cases of amyloid angiopathy have been reported in which dementia was preceded by dysarthric speech, ataxia and/or spasticity of the legs. We assume that these cases represent a distinct nosological entity, different from a variant course of Alzheimer's disease. The atypical symptomatology may cause problems in the diagnosis of amyloid angiopathy of the CNS.