title: Disruption of nigrostriatal and cerebellothalamic pathways in dopamine responsive Holmes' tremor.
abstract: Holmes' tremor is an unusual combination of rest, postural and kinetic tremor of the extremities. Medical treatment of this condition still remains unsatisfactory. The case of a 20-year-old female patient is reported who developed right-sided Holmes' tremor 9 months after a left-sided, cavernoma induced midbrain/pontine haemorrhage at the age of 16 years. Beta-CIT single photon emission computed tomography revealed abolished dopamine transporter activity in the left basal ganglia and striatum, in accordance with missing ipsilateral tegmento-frontal connectivity (medial forebrain bundle), demonstrated by diffusion tensor MRI. Tractography showed reduced fibre connectivity of the superior and middle cerebellar peduncles on the lesioned side. Administration of pramipexole and L-DOPA led to a clinically significant reduction in tremor severity. In conclusion, our results support the notion that Holmes' tremor was a result of diminished striatal dopaminergic input in our patient.
authors.affiliation: Department of Neurology, Medical University Vienna, Währinger Gürtel 18-20, 1090 Vienna, Austria. stefan.seidel@meduniwien.ac.at
authors.forename: S
authors.initials: S
authors.lastname: Seidel
authors.forename: G
authors.initials: G
authors.lastname: Kasprian
authors.forename: F
authors.initials: F
authors.lastname: Leutmezer
authors.forename: D
authors.initials: D
authors.lastname: Prayer
authors.forename: E
authors.initials: E
authors.lastname: Auff
mesh_terms: D052160:Benzothiazoles / Q000627:therapeutic use; D002531:Cerebellum / Q000000981:diagnostic imaging / Q000473:pathology*; D002543:Cerebral Hemorrhage / Q000473:pathology; D038524:Diffusion Magnetic Resonance Imaging; D015259:Dopamine Agents / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007980:Levodopa / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D017072:Neostriatum / Q000000981:diagnostic imaging / Q000473:pathology*; D009434:Neural Pathways / Q000000981:diagnostic imaging / Q000473:pathology*; D000077487:Pramipexole; D013378:Substantia Nigra / Q000000981:diagnostic imaging / Q000473:pathology*; D013577:Syndrome; D013788:Thalamus / Q000000981:diagnostic imaging / Q000473:pathology*; D015899:Tomography, Emission-Computed, Single-Photon; D014202:Tremor / Q000000981:diagnostic imaging / Q000473:pathology*; D055815:Young Adult
chemical_list: D052160:Benzothiazoles; D015259:Dopamine Agents; D007980:Levodopa; D000077487:Pramipexole