Search

Objectives Reduced cardiac meta -iodobenzylguanidine (MIBG) uptake and loss of cardiac sympathetic axons, as its possible anatomical substrate, were both recognised in Lewy body disease (LBD), while their direct correlation has so far remained speculative. Increasing availability of autopsy-confirmed cases of LBD prompted us to quantify residual cardiac sympathetic axons to establish their relationship to cardiac MIBG uptake. Methods We collected cardiac tissue samples from 23 patients with autopsy-confirmed LBD and two non-LBD control patients who underwent 123 I-MIBG cardiac scintigraphy in life. Samples of the left ventricular anterior wall were stained with anti-tyrosine hydroxylase (TH) and anti-neurofilament (NF) antibodies as markers of cardiac nerve axons. We quantified the immunolabelled areas and assessed their correlation to standardised heart to mediastinum (H/M) ratios of 123 I-MIBG cardiac scintigraphy. Results Cardiac MIBG uptake in the early and delayed phases was reduced in 90.9% and 95.7% of patients with LBD, respectively. The area of TH-immunoreactive axons correlated significantly with the H/M ratio in the early (p=0.036) as well as in the delayed (p=0.018) phases. The area of NF-immunoreactive axons also correlated with the H/M ratio in the early (p=0.003) as well as in the delayed (p=0.001) phases. Conclusions Tight quantitative correlation between cardiac 123 I-MIBG uptake and corresponding loss of sympathetic axons in LBD, as established for the first time by this study, provides a scientific basis to confirm the reliability of MIBG cardiac scintigraphy as a powerful clinical tool to detect loss of these axons as a biomarker for the presence of Lewy body disease.

Orthostatic hypotension sometimes is a reversible neurological complication of vitamin B12 deficiency.1 2 Eisenhofer detected deficient sympathetic catecholamine release in insulin tolerance testing,2 but the mechanism of orthostatic hypotension in vitamin B12 deficiency remains unclear. We report a patient with vitamin B12 deficiency and reversible orthostatic hypotension, and discuss the mechanism of this symptom. A 77 year old man admitted to our hospital had had unstable gait and urinary urgency for 6 months, clumsiness of the hands and tingling sensations in the legs for 3 months, and, for a month, occasional dizziness on standing. The dizziness was mild without any attack of syncope. He had no other symptoms or signs of autonomic dysfunction but impotence and erectile failure were noted 10 years before the onset of neurological symptoms. He had not taken any medicine which would affect the autonomic nervous system. He did not have a habit of drinking. Physical examination on admission detected no signs of anaemia, heart failure, or dehydration. …