Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 60

Brachial amyotrophic diplegia (BAD) is defined the isolated upper limb weakness attributed to neurogenic atrophy, sparing lower extremity, respiratory and bulbar muscles. Although such phenotypes may be seen in number of conditions, previous reports pointed out that the label of BAD should refer to any patient with slowly and nonprogressive neurogenic atrophy of upper limbs remaining stable at least 18 months after initial presentation 50 year old man with unremarkable past history noticed after minor cervical spine trauma progressive weakness and wasting of left shoulder and arm which was followed within few months by gradual involvement of contralateral in relatively symmetric pattern. There were no symptoms in lower limbs, neck, bulbar muscles nor sphincter disturbances. On examination, patient exhibited a peculiar posture of both hands hanging loosely at his sides. There were fasciculations and atrophy of upper limb muscles. Reflexes could not be elicited in upper extremities but they were normal in the lower. Cranial nerves and sensory examination were unremarkable. Routine blood tests and search for GM1 antibodies were negative, whereas CK was found mildly elevated ( 361 mU/ml). Test for HIV-1 was positive with RNA level of 144.920 copies/ml and CD4 count 403/mm3. Electrophysiology showed in both sides low amplitude of compound muscle action potentials, mildly reduced velocity without evidence of conduction block or of temporal dispersion. Sensory conduction study was normal. On EMG, there was loss of motor units, acute and chronic neurogenic changes. Cervical MRI was normal. Patient neurological disability did not show progression. The case reported exhibits the following notable aspects: clinical and electrophysiological abnormalities fully restricted to upper limbs, lack of significant demyelinating features as well as of GM1 antibodies, absence of clinical signs related to immunosuppression due to HIV-1 infection.