In vivo assessment of neurodegeneration in Spinocerebellar Ataxia type 7.

Spinocerebellar Ataxia type 7 (SCA7) is a neurodegenerative disease characterized by progressive cerebellar ataxia and retinal degeneration. Increasing loss of visual function complicates the use of clinical scales to track the progression of motor symptoms, hampering our ability to develop accurate biomarkers of disease progression, and thus test the efficacy of potential treatments. We aimed to identify imaging measures of neurodegeneration, which may more accurately reflect SCA7 severity and progression.

A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

Background: Many different oligosynaptic reflexes are known to originate in the lower brainstem which share phenomenological and neurophysiological similarities.

Objective: To evaluate and discuss the differences and aberrancies among these reflexes, which are hard to discern clinically using neurophysiological investigations with the help of a case report.

Methods: We describe the clinical and neurophysiological assessment of a young man who had a childhood history of opsoclonus-myoclonus syndrome with residual mild ataxia and myoclonic jerks in the distal extremities presenting with subacute onset total body jerks sensitive to sound and touch (in a limited dermatomal distribution), refractory to medications.

Objective predictors of 'early tolerance' to ventral intermediate nucleus of thalamus deep brain stimulation in essential tremor patients.

Objective: To identify pre-operative clinical and computerized spiral analysis characteristics that may help ascertain which patients with Essential Tremor (ET) will exhibit 'early tolerance' to ventral intermediate nucleus of thalamus (Vim) deep brain stimulation (DBS).

Methods: Identification of comparative characteristics of defined cases of 'early tolerance' versus patients with sustained satisfactory response treated with Vim DBS surgery for medically-refractory ET, based on retrospective chart review by a clinician blinded to the findings of computerized spiral analysis.

Results: Statistically significant differences in two spiral analysis indices, SWVI and DoS, were found in the dominant upper limbs of patients who developed 'early tolerance', whereas the clinical characteristics were not significantly different.

Cervical angina: an overlooked source of noncardiac chest pain.

Cervical angina has been widely reported as a cause of chest pain but remains underrecognized. This series demonstrates the varied clinical presentation of patients with cervical angina, the delay in diagnosis, and the extensive cardiac examinations patients with this condition typically undergo prior to a definitive diagnosis. Recognition of this condition in patients with acute chest pain requires a high index of suspicion and an awareness of the common presenting features and clinical findings of cervical angina.