Neurol. Int., Volume 11, Issue 2 (June 2019) – 7 articles

Chronic migraine is a frequent and debilitating condition affecting 14% of the general population. This prospective observational pilot study investigated whether men with chronic migraine have lower than expected total serum testosterone levels. We identified 14 men ages 26-51 at our Institution who fulfilled the ICHD-3b criteria for chronic migraine and obtained serum total testosterone levels. The mean total testosterone level in our 14 patients was 322 ng/dL (range: 120-542 ng/dL) which is in the lower 5% of the reference range for our laboratory (300-1080 ng/dL). Men with chronic migraine had lower total testosterone levels compared to published agematched normative median values by a median difference of 62 ng/dL (P=0.0494). This finding suggests that hypothalamic regulation is altered in patients with chronic migraine. Further studies are warranted to determine whether testosterone supplementation in men with chronic migraine reduces the number of headaches or the associated symptoms of hypogonadism. Full article

Andersen-Tawil syndrome (ATS) is characterized by a triad of periodic paralysis, cardiac arrhythmias and distinctive dysmorphic features. Due to its rarity and high degree of clinical and phenotypic variability, a diagnosis of ATS can be very perplexing and challenging. Herein, an atypical case of ATS with a complicated presentation that caused an approximately 11-year delay in diagnosis is reported. The patient made a full recovery with acetazolamide after the diagnosis. The case and its management are presented with an updated literature review. Full article

This study aims to evaluate the prevalence, distribution and clinical profile of neurological diseases and syndromes from a neurology outpatient clinic in the city of Rio de Janeiro. This is a quantitative, cross-sectional study. 180 medical records of firsttime visits conducted through 18 months were analyzed. Age, gender and comorbidities were collected to identify the sample’s profile and the neurological diagnoses found were classified in predefined groups of syndromes according to the ICD-10 classification, which were further stratified according to age and gender. 157 (87,2%) of the patients had a neurological disease, of which the mean age was 51,05 years and 67,5% were women. The most frequent comorbidities were: hypertension (55,6%); dyslipidemia (20,4%); and diabetes (14,6%). The most common neurological diseases were: Sequelae of Cerebrovascular Disease (12,7%); Unspecified Dementia (8,9%); and Migraine without Aura (7,6%). The main neurological syndromes were: Headache (24,8%); Dementia (15,9%); Cerebrovascular Disease (13,4%); Movement Disorders (12,1%); and Radiculopathy (10,8%). The age and gender profile of these syndromes was further described. These data contribute to better understand the distribution of neurological diseases in the neurological outpatient setting. Full article

We present the case of a 44-year-old man with amyotrophic lateral sclerosis (ALS) intubated for hypercapnic respiratory failure and aspiration pneumonia. The patient was successfully extubated, transitioned to non-invasive ventilation and lived at home comfortably for 17 months, with good functional status for the first year. This case highlights the potential of prolonged survival post extubation in patients with advanced ALS and respiratory failure. The patient was managed post-discharge in a multidisciplinary ALS clinic by experienced neuromuscular and pulmonary specialists. Full article

We describe the case of a young man, 22 years old, a household contact of leprosy, presenting paresthesia in the right leg and great thickening in the anterior femoral nerve and in the ipsilateral sural nerve. There were no typical dermatological lesions of leprosy. Ultrasonography confirmed the clinical finding. The clinical diagnosis of the disease was established and the patient started multibacillary-multidrug therapy, obtaining improvement of the initial symptomatology. Full article

Pompe disease (PD) is a rare neuromuscular disorder caused by a deficiency of the enzyme acid alpha-glucosidase. There are three forms of PD depending on the age at onset and clinical severity. PD causes involvement of different organ systems, such as the heart, musculoskeletal system, and respiratory system. As of today, enzymere placement therapy represents the main therapeutic tool for PD. Rehabilitation is an integral part of a multidisciplinary approach to this pathology. The goal of the present review is to find scientific evidence for the rehabilitative approach to PD, with respect to both the infantile- and adult-onset forms. A systematic literature review was made using the following databases: Pubmed, Pedro, Cochrane Library, EDS Base Index, Trip, and Cinhal. Randomized controlled trials or cohort studies with a sample population of at least six subjects were retrieved. The PICO method was used to formulate the clinical query. The search resulted in 1665 articles. Of these, four cohort studies were subjected to the final phase of the review. Three studies regarded inspiratory muscle training with a threshold, while the fourth study analyzed the effectiveness of therapeutic, aerobic, and reinforcement exercises. Inspiratory muscle training with a threshold increases the pressures generated during inhalation. Aerobic exercise is capable of increasing patients’ muscular endurance and performance. To date, however, rehabilitative treatment for patients with PD has no validation in evidence-based medicine. Further studies, possibly with a larger sample size and higher quality are necessary to confirm the effectiveness of rehabilitation in patients with PD. Full article

Large cerebral infarctions have high morbidity and mortality. Patients with large cerebral infarctions may have recurrent ischemia as high as 8.1% within 7 days; highest among other types of strokes. Data regarding risk factors for large cerebral infarction in Asian populations are still scant. All adult (age ≥15 years old) patients with the diagnosis of thrombotic ischemic stroke who were treated at Srinagarind Hospital, Khon Kaen University, Thailand from January 2012 to December 2013 were studied. Large cerebral infarctions are defined by clinical criteria of having cerebral cortical impairment, brain stem or cerebellar dysfunction with infarction sizes of more than 1.5 cm. The association of various stroke risk factors and large infarction strokes were calculated using multiple logistic regression analysis. There were 276 thrombotic stroke patients who met the study criteria; classified as large cerebral infarctions in 59 patients (21.38%) and small cerebral infarctions in 217 patients (78.62%). Baseline characteristics and risk factors for stroke were comparable between both groups. The large cerebral infarction group had a significantly larger proportions of right internal carotid artery stenosis, plaques on the left side, left internal carotid artery stenosis, and internal carotid artery stenosis at any side than the small cerebral infarction group. Among various stroke risk factors, only internal carotid artery stenosis at any side was the only significant factor associated with large cerebral infarction with an adjusted odds ratio of 11.14 (95% CI: 3.46, 35.82). In conclusion, significant internal carotid artery stenosis is associated with large cerebral infarction. Full article