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Abstract Details

Secondary Blepharospasm Associated with Structural Lesions of the Brain
Movement Disorders
P02 - (-)
062
BACKGROUND: Blepharospasm is a form of focal dystonia manifest as involuntary repetitive spasms of the orbicularis oculi muscles. Dysfunction of basal ganglia is traditionally presumed to be the main cause of blepharospasm. However, recent evidence suggests that a network of multiple cortical and subcortical structures is involved.
DESIGN/METHODS: With IRB approval, electronic medical records at Emory were queried to identify patients with blepharospasm from 2001 to 2011. Medical records including physician notes and radiology reports were reviewed and demographic and imaging data were extracted. Additionally, a systematic review of the literature was done to find cases of secondary blepharospasm reported through June 2012.
RESULTS: A total of 996 patients were treated for blepharospasm at Emory, 156 with imaging data. Eighteen patients were identified with a lesion. Lesions were located in thalamus (n=5), cerebellum (n=4), brain stem (n=3) midbrain (n=3), basal ganglia (n=2), and parietal cortex (n=1). Twenty six articles reporting 31 cases of secondary blepharospasm were found in review of the literature from 1983 to 2012. Among these, lesions were located in the thalamus (n=7), basal ganglia (n=7), brainstem (n=7), cerebellum (n=5), midbrain (n=4), lateral ventricle (n=1), and cortex (n=2). In 4 patients, no discrete lesion was reported and imaging findings were documented as diffuse atrophy. For this summary, some cases with lesions in more than one region were counted more than once.
CONCLUSIONS: The basal ganglia are not the only region involved in the pathogensis of secondary blepharospasm. Instead, the available data support a network model, which proposes involvement of multiple areas. Abnormal function or communication between parts of this network causes irregularities in motor control manifesting as dystonia.
Authors/Disclosures
Mohammad Khoshnoodi, MD (Tower Health)
PRESENTER
No disclosure on file
Stewart A. Factor, DO, FAAN (Emory University School of Medicine) Dr. Factor has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Neurocrine. Dr. Factor has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Factor has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda. The institution of Dr. Factor has received research support from Biohaven. The institution of Dr. Factor has received research support from Neurocrine. The institution of Dr. Factor has received research support from Supernus. The institution of Dr. Factor has received research support from Sun Pharmaceuticals Advanced Research Company. The institution of Dr. Factor has received research support from Aspen. The institution of Dr. Factor has received research support from RHO. Dr. Factor has received publishing royalties from a publication relating to health care. Dr. Factor has received publishing royalties from a publication relating to health care. Dr. Factor has received publishing royalties from a publication relating to health care. Dr. Factor has received publishing royalties from a publication relating to health care.
H. A. Jinnah, MD, PhD, FAAN (Emory University) Dr. Jinnah has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takaha/Ene. Dr. Jinnah has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Ipsen. Dr. Jinnah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ipsen. Dr. Jinnah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Abbvie. The institution of Dr. Jinnah has received research support from Addex. The institution of Dr. Jinnah has received research support from Aeon. The institution of Dr. Jinnah has received research support from Revance. The institution of Dr. Jinnah has received research support from Jazz.
Edythe Boitnott No disclosure on file