Case Of the Week (COW) 18 Jan 2015
Answer:
Hyperglycemic Hemichorea-Hemiballismus(HCHB syndrome)
Findings:
Right caudate and basal ganglia hyperintensity on T1.
Discussion:
Triple H of Hyperglycemic Hemichorea-Hemiballismus : Unilateral basal ganglionic T1 Hyper intensity Hyper glycemia / Hyper glycemic coma Hemi chorea / Hemi ballismus It is a syndrome associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus, characterized by sudden onset hemiballismus or hemichorea. The most common cause of hemichorea hemiballism in adults is a vascular lesion in the basal ganglia. Rarely, it can also be the first clinical manifestation of non-ketotic hyperglycemia, associated with unique radiological features.
Imaging findings of Hyperglycemic Hemichorea-Hemiballismus: CT may be normal. May show faint unilateral basal ganglionic hyperdensity. MRI is most sensitive. May show typical unilateral T1 hyperintensity in basal ganglia. Chang and colleagues postulated petechial haemorrhage to be the cause. Stereotactic biopsy and histopathology from the striatum revealed gliotic brain tissue with abundant gemistocytes suggesting that the hyperintensities in T1 could be due to the protein hydration layer inside the cytoplasm of the swollen gemistocytes. These gemistocytes abundantly present in the basal ganglia and cause excessive neuronal activity especially in the GABA-ergic projections and thus may be responsible for generating hemichorea-hemiballism.The basal ganglia hyperintensity generally resolves within a few months rarely reported to remain for several years.
Contributed By:
Dr. Balaji Ayyamperumal
Tanjore medical college
Answer:
Hyperglycemic Hemichorea-Hemiballismus(HCHB syndrome)
Findings:
Right caudate and basal ganglia hyperintensity on T1.
Discussion:
Triple H of Hyperglycemic Hemichorea-Hemiballismus : Unilateral basal ganglionic T1 Hyper intensity Hyper glycemia / Hyper glycemic coma Hemi chorea / Hemi ballismus It is a syndrome associated with nonketotic hyperglycemia in patients with poorly controlled diabetes mellitus, characterized by sudden onset hemiballismus or hemichorea. The most common cause of hemichorea hemiballism in adults is a vascular lesion in the basal ganglia. Rarely, it can also be the first clinical manifestation of non-ketotic hyperglycemia, associated with unique radiological features.
Imaging findings of Hyperglycemic Hemichorea-Hemiballismus: CT may be normal. May show faint unilateral basal ganglionic hyperdensity. MRI is most sensitive. May show typical unilateral T1 hyperintensity in basal ganglia. Chang and colleagues postulated petechial haemorrhage to be the cause. Stereotactic biopsy and histopathology from the striatum revealed gliotic brain tissue with abundant gemistocytes suggesting that the hyperintensities in T1 could be due to the protein hydration layer inside the cytoplasm of the swollen gemistocytes. These gemistocytes abundantly present in the basal ganglia and cause excessive neuronal activity especially in the GABA-ergic projections and thus may be responsible for generating hemichorea-hemiballism.The basal ganglia hyperintensity generally resolves within a few months rarely reported to remain for several years.
Contributed By:
Dr. Balaji Ayyamperumal
Tanjore medical college