Desafios diagnósticos na Síndrome da Taquicardia Postural Ortostática (POTS) em uma adolescente / Challenges on the diagnostis of Postural Orthostatic Tachycardia Syndrome (POTS) in a adolescent patient

Descreveu-se a Síndrome da Taquicardia Postural Ortostática (POTS) em uma adolescente de 16 anos, com história de 10 anos de hipotireoidismo e episódio de síncope incompleta e sintomas disautonômicos flutuantes por seis meses. Após esses seis meses, a paciente apresentou novo episódio de síncope incompleta pós-prandial. Apesar de um Tilt Test não ter flagrado anormalidade, em duas ocasiões separadas, flagramos aumentos ortostáticos da frequência cardíaca de 43 e 40 bpm, sem queda significativa da pressão arterial. Além de hipotireoidismo, não se detectaram outros fatores de risco para disautonomia. Propranolol (10mg, 12/12h) controlou os sintomas. POTS é de difícil. (AU)

We describe the Postural Orthostatic Tachycardia Syndrome (POTS) in a 16-year-old girl with a 10-year history of hypothyroidism who experienced an incomplete syncope episode with fluctuating disautonomic symptoms for 6 months. After six months, the patient had a new postprandial incomplete syncope episode. Although a Tilt Test did not disclose abnormality, in two separate occasions, we documented a heart rate increase of 43 and 40 bpm after standing, without a significant drop in blood pressure. Other than hypothyroidism, we did not detect other risk factors for dysautonomia. Propranolol (10mg, 12 / 12h) controlled the symptoms. POTS is difficult to diagnose in adolescents. It is often confused with Epilepsy and Neurocardiogenic Syncope, which may result from hypothyroidism. (AU)

Small (autonomic) and large fiber neuropathy in Parkinson disease and parkinsonism.

BACKGROUND: Recent studies have reported that peripheral neuropathy (PN) is common in patients with Parkinson's disease (PD) and raised the possibility that levodopa neurotoxicity is the main culprit. METHODS: We evaluated the presence of large & small (autonomic) fiber PN in 54 consecutive patients with PD or parkinsonism in a tertiary outpatient clinic from Brazil. Initial PN screening consisted of history/neurological exam and skin wrinkling test (SWT). In addition, we also performed Nerve conduction studies/Electromyography (NCS/EMG) in all patients with PN signs/symptoms and/or abnormal SWT. RESULTS: Thirty eight patients with PD (10 women, mean age: 63 ± 2.1 years, P < 0.05 versus parkinsonism, mean disease duration: 8 ± 0.8 years) and 16 patients with other forms of parkinsonism [7 women, mean age: 50.1 ± 3.9 years, mean disease duration: 6.9 ± 1.1 years] completed clinical neuromuscular evaluation. SWT was performed in 48 patients (33 PD, 15 parkinsonism). In the PD group, SWT was abnormal in 57.6% of the tested patients (comprising 50% of all PD patients). In the parkinsonism group, SWT was abnormal in 37.5% (comprising 35.3% of all parkinsonism patients). NCS/EMG was performed in 39 patients (26 PD and 13 parkinsonism). Twelve out of the 26 PD (34.2% of all PD) and 4 out of the 13 parkinsonism (23.5% of all parkinsonism) had abnormal NCS/EMG results. Neuropathy prevalence was similar in PD and parkinsonism groups as detected either by NCS/EMG or SWT. CONCLUSIONS: Large fiber and small (autonomic) fiber PN are common in patients with PD and parkinsonism. The etiology for the neuropathy was likely to be multifactorial and may be secondary to PD itself.