Quality of neurology residency programmes: an Italian survey.

In recent years there has been a growing interest in medical and particularly neurological education and how this should be related to the needs for patient care. To evaluate neurological training in Italy, we conducted a survey of the residency programmes aimed at different aspects of training. The survey was conducted in the 38 neurological Italian teaching hospitals and 27 of these answered. Six of the 27 centres organized all of the scheduled teaching courses. The quality of courses was considered 'not sufficient' in 11 schools and 'good' in 12. Seminars were regularly performed in 18 centres but in 60% of these the number was <1 per week. Questionnaires to evaluate the quality of teaching were lacking in all centres. Regarding the procedures performed by each resident there was a large variation between the different schools. A regular rotation of each resident in the neurophysiology services was performed in 14 schools. Ward and out patient activity varied widely and details are given. We conclude that there is marked heterogeneity in training programmes between different centres. Some important activities such as seminars and rotation in neurophysiology are performed poorly.

Supplemental therapy in isolated vitamin E deficiency improves the peripheral neuropathy and prevents the progression of ataxia.

A 24-year-old male, who suffered since childhood from a progressive form of ataxia associated with peripheral neuropathy, was found severely deficient in serum vitamin E. He walked with bilateral aid and presented severe dysmetria of the limbs and dysarthric speech; muscular strength and trophism were slightly diminished in the distal muscles of four limbs and there was hypotonia of the arms; he presented absent deep tendon reflexes, bilateral Babinski's sign, reduced proprioception at four limbs, pes cavus and fasciculations of the tongue. Intestinal fat malabsorption and other gastrointestinal or haematological conditions associated with deficiency of this vitamin were ruled out. In this patient, after 2 years of a daily supplement of high doses of vitamin E, a further progression of the disease was not observed and, moreover, the neurophysiological characteristics of his neuropathy appeared clearly improved. A longitudinal evaluation of serum vitamin E levels showed values in the normal range after 13 months of therapy. The patient had molecular genetic analysis of chromosome 8 and was found homozygous for the unusual mutation 513insTT in the alpha-tocopherol transfer protein gene.