Isolated bulbar paralysis in a patient with medullar tau pathology: a case report.

Increased morbidity and mortality rates as well as some other manifestations in Alzheimer's disease can be explained by subcortical degeneration. Pathological evidence is scarce but confirmative. We report a 77-year-old patient who presented with a 6-year history of dyspnoea, stridor and dysphagia. Unexpectedly, histopathological examination disclosed extensive degeneration of the medulla by tau pathology. The majority of symptoms and signs could be explained by the medullary tau pathology. Whether the medullary tau pathology in this case was a rare aberrant progression of Alzheimer's disease or a new presentation of tauopathy concomitant with subclinical Alzheimer's disease should be elucidated by additional studies.

Autonomic dysfunction in presymptomatic and early symptomatic Huntington's disease.

OBJECTIVES: Although autonomic dysfunction was found in advanced Huntington's disease (HD) patients it is not clear whether there is autonomic dysfunction in presymptomatic and early symptomatic HD. MATERIAL & METHODS: Different cardiovascular autonomic tests were performed in 14 presymptomatic HD mutation carriers (PHD), 11 early symptomatic HD patients (EHD) and in 25 sex and age matched controls. RESULTS: We found attenuated response to simple mental arithmetic test (relative heart rate in PHD and EHD was 10% lower than in controls; diastolic pressure was 10.6% lower in EHD than in controls; P < 0.05) and exaggerated response to the late phase of cold pressor test (relative heart rate was 10% higher in PHD and 7% higher in EHD than in controls; P < 0.05). The rest of the cardiovascular autonomic tests did not reveal significant differences between patients and controls. CONCLUSIONS: Our results suggest that subtle autonomic dysfunction occurs even in PHD and EHD.

Incipient cardiovascular autonomic imbalance revealed by wavelet analysis of heart rate variability in Type 2 diabetic patients.

AIM: Incipient cardiovascular autonomic imbalance is not readily diagnosed by conventional methods. Spectral analysis of heart rate variability (HRV) by wavelet transform (WT) was used to measure cardiovascular autonomic function in patients with Type 2 diabetes. METHODS: Thirty-two diabetic patients without (D), 26 with cardiovascular autonomic neuropathy (DAN) and 72 control subjects (C) participated. A 30-min HRV time series was analysed by wavelet transformation and four characteristic frequency intervals were defined: I (0.0095-0.021 Hz), II (0.021-0.052 Hz), III (0.052-0.145 Hz) and IV (0.145-0.6 Hz). RESULTS: When compared with C, in both D and DAN the normalized power and amplitude of interval II were increased and of interval IV decreased, resulting in a significantly higher II/IV ratio. Furthermore, in DAN the normalized power and amplitude of interval I were increased and of interval III decreased when compared with the D and C groups. The diabetic patients were divided in two equal subgroups according to HbA(1c) < 8.0% and >or= 8.0%. In the subgroup with HbA(1c) >or= 8.0%, normalized power in interval II was significantly higher and in interval IV significantly lower than in the subgroup with HbA(1c) < 8.0%. In D, but not in DAN patients prescribed ACE inhibitors, the absolute amplitude and power of oscillations were significantly higher than in patients not taking ACE inhibitor therapy. CONCLUSIONS: Patients with diabetes have increased sympathetic and decreased parasympathetic cardiac activity regardless of the presence of autonomic neuropathy. Glycaemic control and treatment with ACE inhibitors may favourably influence HRV in diabetic patients without autonomic neuropathy.

Early sympathetic hyperactivity in Huntington's disease.

To investigate whether Huntington's disease (HD) affects autonomic nervous system (ANS) functioning 33 subjects with positive genetic test results were studied. The subjects were classified according to Shoulson and Fahn (S&F) HD disability scale into three subgroups: subgroup 1 (eight asymptomatic gene carriers), subgroup 2 (13 mildly disabled HD patients) and subgroup 3 (eight moderately and four severely disabled HD patients). A battery of cardiovascular autonomic tests (Valsalva maneuver, deep breathing test, sustained handgrip test, orthostatic test) and the spectral analysis of heart rate variability (HRV) were performed. The results were compared with a group of matched controls. In subgroup 1, there was a higher power of low-frequency band (LFB) (P < 0.05). In subgroup 2 a higher power of LFB was detected, LFB/high-frequency band (HFB) coefficient was increased and the blood pressure response to sustained handgrip was elevated (P < 0.05). Subgroup 3 showed significantly lower blood pressure response to sustained handgrip, lower respiratory (P < 0.05) and orthostatic ratio (P < 0.01). Our results suggest that sympathetic hyperfunction is present in asymptomatic gene carriers and mildly disabled HD patients. Contrary to that, ANS hypofunction was found in advanced HD patients.

Beat-to-beat QT interval variability before and after cardiac surgery.

Non-uniform recovery of excitability may be essential in triggering malignant ventricular tachycardia after cardiac surgery. Thirty-five channels ECG was recorded for 6 min in 27 patients before and after heart surgery and in 20 control subjects. Off-line analysis was performed. RR interval duration, RR SD, QT SD and power spectra of RR variability were computed from 256 s stable RR and QT interval series. When compared to controls, patients had decreased RR SD and increased QT SD before surgery (p<0.002 and p<0.0005, respectively); RR SD further decreased and QT SD increased after the surgery (p<0.0001 and p<0.0002, respectively). Increase of QT variability and decrease of RR variability after cardiac surgery may reflect disrupted electrophysiological stability of the myocardium and thus electrophysiological substrate for triggering malignant arrhythmia.

Anti-Yo-associated paraneoplastic cerebellar degeneration in a man with gastric adenocarcinoma.

Paraneoplastic cerebellar degeneration (PCD) with anti-Yo antibodies is almost always associated with ovarian and breast cancer. We describe a man with anti-Yo-positive PCD and gastric adenocarcinoma. The tumor cells were labeled with anti-Yo antibodies by immunohistochemistry. High serum titers of anti-Yo antibodies were found before surgery and decreased 6 months after resection of the tumor. The expression of Yo antigens by the tumor and the decrease in anti-Yo antibody titers after its removal suggest that the immune response against the Purkinje cells of the cerebellum was triggered by the tumor.

Autonomic nervous system function in patients with acute brainstem stroke.

Dysfunction of the autonomic nervous system (ANS) often complicates the clinical course in patients with acute stroke. The studies of the function of ANS in patients with brainstem stroke are scarce. The purpose of our study was to evaluate the function of ANS in the early period after acute brainstem stroke and to find out whether the location of stroke in brainstem influences the function of ANS. We studied heart rate variability (HRV) and plasma levels of catecholamines in 14 eligible patients with medullary (6 patients) and non-medullary (8 patients) brainstem stroke during the first 6 weeks after stroke. Integrals over the low- (LFB; 0.04-0.15 Hz) and high-frequency bands (HFB; 0.15-0.40 Hz) of power spectra were calculated. When compared to controls, the integrals over HFB in the hyperacute (p < 0.001) and over LFB in the hyperacute (p < 0.005) and in the acute (p < 0.05) phases were significantly smaller in patients with medullary strokes. Integrals over LFB and HFB in patients with non-medullary strokes did not differ significantly from controls, regardless of the phase of the disease. Plasma levels of epinephrine in patients with non-medullary stroke in the hyperacute and in the acute phases were significantly higher than in controls (p < 0.005). We conclude that there is transient dysfunction of ANS in patients with acute medullary stroke, in contrast to those with non-medullary brainstem stroke.