ABSTRACT

This chapter provides that the themed presentation encourages quick, focused study and detailed answers aid comprehension and encourages familiarity with each topic with essential diagrams, colour images and sample MRIs. Symptoms usually begin in childhood, characterised by a slowly progressive ataxia and associated with weakness, scoliosis, bladder dysfunction, hypo/a-reflexia, dysarthria. Demyelination of the dorsal columns can also cause loss of proprioception and vibration sense. Cardiomyopathy and diabetes are also commonly associated with Friedreich’s ataxia. Binswanger’s disease is a small vessel vascular dementia that results in memory loss and decline in cognitive ability. White matter lesions are thought to occur due to atherosclerosis. Management is usually symptomatic with analgesia and involves a multidisciplinary team approach including orthopaedic surgeons for correction of severe pes cavus deformities.