TY - JOUR T1 - Sensory ataxia as the initial clinical symptom in X-linked recessive bulbospinal neuronopathy JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 277 LP - 277 DO - 10.1136/jnnp.69.2.277 VL - 69 IS - 2 AU - ANSGAR BUECKING AU - ROBERT PFISTER Y1 - 2000/08/01 UR - http://jnnp.bmj.com/content/69/2/277.1.abstract N2 - X-Linked recessive bulbospinal neuronopathy (X-BSNP) has previously been described as a disease in which the first clinical symptoms which occur concern the motor system. A weakness of the shoulder and pelvic girdle muscles as well as cramps and muscle pain in the proximal limbs are normally found in the early stages.1-3 The onset of X-BSNP generally ranges between the ages of 25 and 50 years; the disorder then shows a slow but continuous progression of symptoms.1 3 An involvement of facial and bulbar musculature with fasciculations and atrophy of these muscles and, therefore, often dysarthria and dysphagia, are common symptoms of an advanced stage.1 3 Nevertheless, life expectancy does not seem to be considerably reduced.1Sensory impairment was reported to be minimal or non-existent.1-3 Pathoanatomical studies showed that a degeneration of both the lower motor and primary … ER -