Standard and modified statistical MUNE evaluations in spinal-bulbar muscular atrophy.

Motor unit number estimation (MUNE), a technique used in amyotrophic lateral sclerosis (ALS) clinical trials to quantitatively assess motor neuron loss, should also be valuable in assessing progression in spinal bulbar muscular atrophy (SBMA), an x-linked neuronopathy. In ALS, instability of single motor units (SMUP) prompted Shefner et al.6(6) to modify the statistical MUNE method to exclude SMUPs < or = 40 microV.

Isolated relative afferent pupillary defect secondary to contralateral midbrain compression.

Background: Relative afferent pupillary defects are typically related to ipsilateral lesions within the anterior visual pathways.

Objective: To describe a patient who had a workup for headache and was found to have an isolated left relative afferent pupillary defect without any other neurological findings.

Design: We review the neuroanatomy of the pupillary light reflex pathway and emphasize the nasotemporal bias of decussating fiber projections, which accounts for the relative afferent pupillary defect contralateral to the described lesion.