Cardiac autonomic involvement in Huntington's disease.

INTRODUCTION: Huntington's disease (HD) is known as a neurodegenerative disease with movement disorder and cognitive impairment; autonomic involvement is also becoming common in some recent studies. The aim of this study is to demonstrate the presence of cardiac autonomic involvement in HD patients. METHOD: Time and frequency domain parameters obtained from the 24-h Holter ECG(hECG) were compared between 20 HD patients and 20 healthy control subjects. RESULTS: Fourteen HD patients had tachycardia, bradycardia, and extra beats. Interval between two heartbeats, normal-to-normal (NN), standard deviation of all normal-to-normal (SDNN), square root of the mean of the sum of the squares of the differences between consecutive N-N intervals in ms (rMSSD), and the ratio of the number of consecutive pairs of N-N intervals that differ by more than 50 ms to the total number of N-N intervals (pNN50) were all significantly higher in the patient group than in the control group during 24-h hECG monitoring. However, hECG monitoring showed that the patient group had significantly higher values of the frequency-domain metrics high frequency (HF) than the control group did (P = 0.003). Very low frequency (VLF) was lower in the patient group (P = 0.009). There was no difference in low frequency (LF) in both groups. In comparison to the control group, LF/HF was much reduced in the patient group (P = 0.001). CONCLUSION: Cardiac disfunction increases, and autonomic functions change in HD, but more comprehensive studies are needed to distinguish sympathetic and parasympathetic involvement.

Capecitabine-related neurotoxicity presenting with agraphia.

INTRODUCTION: Capecitabine is a pre-metabolite of 5-fluorouracil and is used as a chemotherapeutic agent. Among the common side effects of capecitabine, there are gastrointestinal side effects including nausea, vomiting, and diarrhea, and dermatological side effects including hand-foot syndrome and skin pigmentation change. However, neurological side effects of capecitabine are very rare. We describe herein a patient who developed neurological side effects in the form of agraphia and dysarthria on the 7th day of capecitabine treatment. CASE REPORT: A 34-year-old male patient, who was being followed up with the diagnosis of colon cancer, presented with speech and writing disorder that developed while under capecitabine treatment. Dysarthria and agraphia were detected in his neurological examination. Diffusion-weighted magnetic resonance imaging (MRI) revealed acute diffusion restriction in the splenium of the corpus callosum and at the level of the bilateral centrum semiovale. Brain MRI revealed symmetrical T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) signal increases at the right temporoparietal medial, corpus callosum level, and bilateral white matter level. MANAGEMENT & OUTCOME: The capecitabine treatment was terminated, and methylprednisolone treatment was administered and plasmapheresis procedure was carried out. Subsequently, significant improvement was observed in the clinical findings and neuroimaging. DISCUSSION: Capecitabine is used as an oral agent; thus, it provides ease of use. Neurological side effects associated with the use of capecitabine reportedly occur very rarely. The findings of this case demonstrated that leukoencephalopathy can be seen during the use of capecitabine, imaging results are very important in the diagnosis of leukoencephalopathy, and improvement can be achieved with the termination of the capecitabine treatment.

The importance of heart rate variability in predicting cardiac autonomic dysfunction in patients with amyotrophic lateral sclerosis.

AIM: Amyotrophic lateral sclerosis (ALS) is a progressive disease characterized by degeneration in the upper and lower motor neurons of the corticospinal tract, brain stem, and spinal cord. Recent studies have revealed that the disease does not present solely with motor neuron involvement. Accordingly, the aim of this study is to investigate the presence of cardiac autonomic impairment in patients diagnosed with ALS. MATERIAL AND METHOD: A total of 61 patients, who were diagnosed with ALS according to the Revised El Escorial Criteria (R-EEC), were included in this prospective study, in addition to the 29 healthy individuals, who were included in the study as controls. In order to assess the cardiac autonomic involvement, the presence of orthostatic hypotension was investigated, and transthoracic echocardiography and 24-hour electrocardiogram (ECG) using a Holter monitor were performed. RESULTS: Orthostatic hypotension was detected in 14 (22.2%) patients. Holter electrocardiogram results of the patient group revealed statistically significantly lower heart rate variability (HRV) indicators in the time domain (SDNN, SDANN, SDNN index, rMSSD, and pNN50) and in the frequency domain (high frequency [HF], low frequency [LF], and very low frequency [VLF]) than those of the control group (P < .05). CONCLUSION: Contrary to the popular opinion, cardiac autonomic dysfunction in patients with ALS can occur at any stage of the disease. Therefore, it is recommended that the patients are evaluated via periodic examinations during the follow-up period for cardiac autonomic involvement.