"Hairy baskets" associated with degenerative Purkinje cell changes in essential tremor.
Essential tremor (ET) is one of the most common neurologic diseases. Increased numbers of torpedoes and Purkinje cell (PC) loss have been documented in the brains of patients with ET. We recently observed a dense and tangled appearance ("hairiness") of the basket cell axonal plexuses that surround PC soma in Bielschowsky preparations of cerebellar cortex in ET brains. Here, we assessed basket cell "hairiness" in 37 ET (32 cerebellar ET; 5 Lewy body variant ET), 21 nondisease control, and 48 disease control brains using a semiquantitative scale. In 8 cerebellar ET cases (25%), there were high basket scores (rating = 3), whereas no Lewy body variant ET, 1 nondisease control (4.8%), and 2 diseased controls (4.2%) had high basket scores (p = 0.001). The hairy basket scores correlated with numbers of torpedoes (p < 0.001) and inversely with numbers of PCs (p = 0.06). Axonal plexus density obtained by image analysis of basket cell processes traced from digitized images was higher in ET than in nondiseased control cases (p = 0.016). Closely spaced sites of synaptic contact between basket cell processes and PCs were identified by electron microscopy in ET cases. These data indicate that structural changes are not restricted to PCs in ET, and that other neurons within their functional network may be involved in its pathogenesis.
GH Sergievsky Center, Columbia University, New York, New York, USA.
Cordelia R
CR
Erickson-Davis
Phyllis L
PL
Faust
Jean-Paul G
JP
Vonsattel
Sachin
S
Gupta
Lawrence S
LS
Honig
Elan D
ED
Louis
D000368:Aged; D000369:Aged, 80 and over; D001369:Axons / Q000473:pathology / Q000648:ultrastructure; D002525:Cerebellar Cortex / Q000473:pathology* / Q000503:physiopathology / Q000648:ultrastructure; D020329:Essential Tremor / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D019044:Image Cytometry; D007395:Interneurons / Q000473:pathology* / Q000648:ultrastructure; D008297:Male; D046529:Microscopy, Electron, Transmission; D009410:Nerve Degeneration / Q000473:pathology* / Q000503:physiopathology; D009433:Neural Inhibition / Q000502:physiology; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology / Q000648:ultrastructure; D017729:Presynaptic Terminals / Q000473:pathology* / Q000648:ultrastructure; D011689:Purkinje Cells / Q000473:pathology* / Q000648:ultrastructure; D016622:Silver Staining