William Adolfo Celso dos Santos

Epidemiology of primary and secondary headaches in a Brazilian tertiary-care center

OBJECTIVE To analyze the demographic features of the population sample, the time of headache complaint until first consultation and the diagnosis of primary and secondary headaches. METHOD 3328 patients were analyzed retrospectively and divided according to gender, age, race, school instruction, onset of headache until first consultation and diagnosis(ICHD-II, 2004). RESULTS Sex ratio (Female/Male) was 4:1, and the mean age was 40.7+/-15 years, without statistical differences between sexes. Approximately 65% of the patients were white and 55% had less than eight years of school instruction. Headache complaint until first consultation ranged from 1 to 5 years in 32.99% patients. The most prevalent diagnosis were migraine (37.98%), tension-type headache-TTH (22.65%) and cluster headache (2.73%). CONCLUSION There are few data on epidemiological features of headache clinic populations, mainly in developing countries. According to the literature, migraine was more frequent than TTH. It is noteworthy the low school instruction of this sample and time patient spent to seek for specialized attention. Hypnic headache syndrome was seen with an unusual frequency.

Sleep Disorders in Machado–Joseph Disease: Frequency, Discriminative Thresholds, Predictive Values, and Correlation with Ataxia-Related Motor and Non-Motor Features

Sleep disorders are common complaints in patients with neurodegenerative diseases such as spinocerebellar ataxia type 3 (SCA3) or Machado–Joseph disease (MJD)—SCA3/MJD. We evaluated the frequency of sleep disorders in SCA3/MJD patients against controls matched by age and gender, and correlated data with demographic and clinical variables. The main sleep disorders evaluated were rapid eye movement (REM) sleep behavior disorder (RBD), restless leg syndrome (RLS), and excessive daytime sleepiness (EDS). We recruited 40 patients with clinical and molecular-proven SCA3/MJD and 38 controls. We used the following clinical scales to evaluate our primary outcome measures: RBD Screening Questionnaire, International RLS Rating Scale, and Epworth Sleepiness Scale. To evaluate ataxia-related motor and non-motor features, we applied the International Cooperative Ataxia Rating Scale, the Scale for the Assessment and Rating of Ataxia, and the Unified Parkinson’s Disease Rating Scale part III. Psychiatric manifestations were tested with the Hamilton Anxiety Scale, and Beck Depression Inventory. The frequency of RBD and RLS were significantly higher in the SCA3/MJD group than in the control group (p < 0.001). There was no difference between both groups with regard to EDS. The accuracy of RDBSQ to discriminate between cases and controls was considered the best area under the ROC curve (0.86). Within-SCA3/MJD group analysis showed that anxiety and depression were significantly correlated with RDB, but not with RLS. Additionally, depression was considered the best predictive clinical feature for RDB and EDS.

Bilateral horizontal gaze palsy with unilateral peripheral facial paralysis caused by pontine tegmentum infarction.

Clinical features of pontine infarction depend on the topography of vascular lesion and most remarkably sometimes the same topographic region can lead to different clinical syndromes (e.g., dorsal pontine tegmentum). In this report we describe an elderly patient with acute dorsal pontine infarction leading to a unique syndrome of bilateral horizontal gaze palsy and unilateral peripheral facial paralysis. We propose that this syndrome could be included as a part of a continuum that involves one-and-a-half syndrome, eight-and-a-half syndrome, and other variants of pontine tegmentum infarction.

Clinical correlates of the restless legs syndrome

OBJECTIVE To determine the clinical correlates of the restless legs syndrome (RLS) in a Brazilian sleep disorders center. METHODS We retrospectively studied 118 patients with RLS from January, 2004, to December, 2010. The analyzed variables were: age at disease onset, gender, race, years of school instruction, primary and secondary RLS, and treatment options. RESULTS Among the studied patients, 83.9% were women with a female/male sex ratio of 5:1. Mean age of the patients at symptom onset ± standard deviation was 41.7±17.9 years-old. The primary RLS was found in 85% of patients. The other 15% remainders consisted of secondary forms, and they were associated with neuropathy, iron deficiency anemia, end-stage renal disease, or Parkinsons disease. Drug therapy for RLS was introduced in 67% of patients. CONCLUSIONS Most patients presented primary RLS with an early disease onset. Further epidemiological studies are welcomed to provide better information on secondary RLS in Brazil.

Stabbing headache as the initial manifestation of herpetic meningoencephalitis

Stabbing headache (SH) is a short-lasting and painful headache that may happen as a primary headache, develop concurrent with other headache types, or may be associated with several conditions [1–3]. We describe a case of a woman who developed a SH as the first manifestation of herpes zoster (HZ) meningoencephalitis. A 79-year-old woman was admitted to the emergency department with a 1 day history of stabbing pain paroxysms in the right temporal and frontal regions lasting a few seconds (the duration of each stab was between 1 and 3 s). The stabs of pain started suddenly and were repetitive and very intense. Physical examination revealed itchy red macules, papules and vesicles on her right chest. Neurological examination showed spatial and temporal disorientation and neck stiffness, without altered consciousness or focal neurological signs. The cerebral spinal fluid (CSF) analysis revealed 106 leukocytes/mm (62% lymphocytes), protein: 63 mg/dL and glucose 52 mg/dL. Endovenous acyclovir treatment was promptly initiated (10 mg/kg/8 hourly). Brain magnetic resonance imaging and electroencephalogram were normal. Varicella zoster virus IgG by enzymelinked immunosorbent assay test in the CSF was positive. After 2 days of acyclovir treatment, the stabs of pain completely disappeared and 60 days after symptoms onset, the patient remained asymptomatic. To our knowledge, this is the first case reporting SH as the initial symptom of HZ meningoencephalitis, alongside with headache improvement after intravenous acyclovir. In previous studies, the authors were able only to report the association with HZ, but no direct relationship between SH and HZ meningoencephalitis [3]. Headache is one of the most frequent symptoms of HZ meningoencephalitis, usually characterized by severe pain [4, 5]. In our case, the headache characteristics were compatible with SH [1] that ameliorated with acyclovir, reinforcing its relationship with an infectious agent. Interestingly, another report showed a patient with herpes simplex encephalitis presenting with a migraine-like headache, which also improved after acyclovir therapy [6]. This case shows the importance of careful evaluation for underlying causes of SH, demonstrating that this uncommon type of headache may be the initial symptom of HZ meningoencephalitis, a potentially life-threatening disease without early recognition and prompt treatment.

Guillain-Barré syndrome associated with H1N1 vaccination

Department of Neurology and Neurosurgery, Federal University of Sao Paulo, Sao Paulo SP, Brazil; Department of Neurology, Hospital e Maternidade Sao Camilo Pompeia, Sao Paulo SP, Brazil; Medical Student, Federal University of Sao Paulo, Sao Paulo SP, Brazil. Guillain-Barre syndrome (GBS) has been described as adverse event following different types of vaccines, such as influenza, varicella, polio virus and hepatitis B. Since 2009 with the emergence of the pandemic H1N1 influenza virus infection in humans, new vaccines have been developed for influenza A (H1N1) as a preventive strategy to reduce transmission, protect groups at increased risk of infection, and decrease complications and death. In this context, we report two adults who developed GBS after H1N1 immunization.

Clinical findings in Creutzfeldt-Jakob disease mimicking dementia with Lewy bodies

Chefe do Setor de Disturbios do Movimento da Universidade Federal de Sao Paulo (UNIFESP), Sao Paulo SP, Brasil.Received 13 February 2008, received in final form 19 June 2008. Accepted 10 July 2008.Dr. Luis Fabiano Marin – Rua Maranhao 192 / 101 - 01240-000 Sao Paulo SP - Brasil. E-mail: luisfabianom@gmail.com

Semantic aphasia as a sole manifestation of acute stroke

Neurology Service, Hospital e Maternidade Sao Camilo (Pompeia), Sao Paulo SP, Brazil: MD, MSc, Neurologist; MD, Neurologist. The concept of aphasia designates impairment in the symbolic domains of language (vocabulary, semantics, phonology, syntax and morphology) by one or more lesions in the dominant cerebral hemisphere, which may be manifest through spoken and written comprehension and production, but can not be explained by motor or sensory deficits in view of the preservation of phono-articulatory structures, and neither by generalized cognitive deficits (consciousness must be preserved). Stroke is the leading cause of aphasia, which may be identified in more than 20% of stroke patients, reaching up to 40% in the acute phase. These language disturbances may help forecast the vascular territories involved in brain injury. Errors in naming are more frequent when associated to grave mistakes in fluency and comprehension both for oral and written material, such as in global aphasia, motor aphasia or sensory aphasia. The diagnosis of semantic aphasia may pass unnoticed if a specific assessment is not undertaken, particularly considering that semantic errors may be produced in naming, reading, spelling, copying or drawing from memory. Prognosis is better than for other vascular aphasic syndromes, but may be related to the size of brain injury (including both the infarcted tissues and the hypoperfused areas) and to the presence of cortical involvement, among other factors. We report the case of a patient who presented with language impairment as the only symptom in the acute stroke phase, leading to a diagnosis of vascular semantic aphasia. The importance of accurate language evaluation for stroke diagnosis will be discussed.

Spontaneous artery dissection in a patient with Human Immunodeficiency Virus (HIV) infection.

BACKGROUND The relationship between human immunodeficiency virus infection and stroke may be attributed in some cases to an underlying vasculopathy such as in spontaneous cervical arteries dissections. CASE REPORT We report the case of an HIV-infected patient who developed a Wallembergs syndrome due to a vertebral artery dissection. Screening laboratory exams showed hyperhomocysteinemia and also high C-reactive protein plasma levels. CONCLUSIONS This is the first case describing the association between arterial dissection (AD) and HIV-infection. We suggest that AD should also be remembered as a possible mechanism of ischemic stroke in HIV-infected patients.