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. 2008 Jan;65(1):101-7.
doi: 10.1001/archneurol.2007.8.

Reduced Purkinje cell number in essential tremor: a postmortem study

Jordan E Axelrad  1 , Elan D Louis, Lawrence S Honig, Ingrid Flores, G Webster Ross, Rajesh Pahwa, Kelly E Lyons, Phyllis L Faust, Jean Paul G Vonsattel
Affiliations

Reduced Purkinje cell number in essential tremor: a postmortem study

Jordan E Axelrad et al. Arch Neurol. 2008 Jan.

Abstract

Background: Clinical and functional imaging evidence suggests that cerebellar dysfunction occurs in essential tremor (ET). In recent postmortem studies, we documented increased numbers of torpedoes (Purkinje cell axonal swellings) in ET patients without Lewy bodies. Purkinje cell loss, however, has never been rigorously assessed.

Objective: To quantitatively assess the number of Purkinje cells in brains of ET patients and similarly aged controls.

Methods: Postmortem cerebellar tissue was available in 14 ET cases (6 with Lewy bodies and 8 without Lewy bodies) and 11 controls. Calbindin immunohistochemistry was performed on paraffin sections of the cerebellum. Images were digitally recorded and blinded measurements of the number of Purkinje cells per millimeter of cell layer (linear density) were made.

Results: Purkinje cell linear density was inversely correlated with age (r= - 0.53, P= .006) and number of torpedoes (r= - 0.42, P= .04). Purkinje cell linear density differed by diagnosis (mean [SD], controls, 3.46 [1.27] cells/mm; ET cases with Lewy bodies, 3.33 [1.06] cells/mm; and ET cases without Lewy bodies, 2.14 [0.82] cells/mm; P= .04), with the most significant difference between ET cases without Lewy bodies and controls, where the reduction was 38.2% (P= .04). In an adjusted linear regression analysis that compared ET cases without Lewy bodies with controls, decreased linear density (outcome variable) was associated with ET (beta= .56, P= .03).

Conclusions: We demonstrated a reduction in Purkinje cell number in the brains of patients with ET who do not have Lewy bodies. These data further support the view that the cerebellum is anatomically, as well as functionally, abnormal in these ET cases.

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Figures

Figure 1
Figure 1
Purkinje cell linear density differed by diagnosis (mean [SD], controls, 3.46 [1.27] cells/mm; essential tremor [ET] cases with Lewy bodies,3.33 [1.06] cells/mm; and ET cases without Lewy bodies, 2.14 [0.82] cells/mm; F=3.71; P=.04), with a 38.2% difference between ET cases without Lewy bodies and controls (P=.04). Bars show SE.
Figure 2
Figure 2
Representative photographs (original magnification ×40) of calbindin immunohistochemistry on similar cerebellar folia sections from 2 individuals: an 84-year-old healthy control (A and B) and a 90-year-old patient with essential tremor (C and D). It is evident that whereas 12 Purkinje cells are seen in the Purkinje layer of the control (A, 4 denoted by arrows), only 2 are seen in the patient with essential tremor (C, arrows). Numerous Purkinje cell bodies are densely stained in the control (B), whereas there are only 2 evident in the patient with essential tremor (D). The Purkinje cell linear density was 4.29 cells/mm in the control and 1.27 cells/mm in the patient with essential tremor.
Figure 3
Figure 3
We stratified essential tremor (ET) cases without Lewy bodies into those with 10 or more torpedoes (n=3) and those with fewer than 10 torpedoes (n=5). Purkinje cell linear density was lower in those with 10 or more torpedoes (mean [SD], 1.42 [0.41] cells/mm or 59.0% lower than that of controls) than in those with fewer than 10 torpedoes (2.57 [0.69] cells/mm or 25.7% lower than that of controls). For comparison of those with 10 or more vs fewer than 10 torpedoes, t=2.59, P=.04. Bars show SE.
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References

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