Tarso Adoni

Recurrent neuromyelitis optica in Brazilian patients: clinical, immunological, and neuroimaging characteristics

Neuromyelitis optica has not been thoroughly studied in Brazilian patients following the discovery of NMO-IgG and its specific antigen aquaporin-4. In this study we aimed to describe the clinical NMO-IgG immunological status and neuroimaging characteristics of recurrent neuromyelitis optica in a series Brazilian patients. We undertook a retrospective study of 28 patients with recurrent neuromyelitis optica, according to 1999 Wingerchuk’s diagnostic criteria. Data on NMO-IgG status, clinical features, and MRI findings were analyzed. Three men and 25 women were evaluated. Median age at onset of disease was 26 years (range 7—55); median time of follow-up was 7 years (range 2—14). The mean time elapsed between the first and the second attack was 17 months (median 8.5; range 2—88). NMO-IgG was detected in 18 patients (64.3%). Four patients died due to respiratory failure. Most patients presented with cervical (36%) and cervical-thoracic myelitis (46.4%). Holocord lesion was the most common pattern of involvement (50%) on the axial plane. We did not find a statistical association between myelitis extension and NMO-IgG result. Our series of Brazilian patients showed a younger age of onset than previously reported. In our series, in contrast to previous reports, there was no correlation between the extension of myelitis and NMO-IgG positivity.

Seroprevalence of NMO-IgG antibody in Brazilian patients with neuromyelitis optica

OBJECTIVE To determine the seroprevalence of neuromyelitis optica antibody (NMO)-IgG in Brazilian patients with clinical diagnosis of relapsing neuromyelitis optica, also known as Devics disease. METHOD We determined NMO-IgG titers in 28 patients (25 of them females) that fulfilled the 1999 NMO diagnostic criteria proposed by Wingerchuk et al. RESULTS NMO-IgG was detected in 18 NMO patients (64.3%). CONCLUSION Our results showed that seroprevalence of NMO-IgG in Brazilian NMO patients was similar to the observed in other studies.

Hypersexuality following bilateral thalamic infarction: case report

Hypersexuality is a rare but well recognized condition following brain injury. It has been described secondarily to dysfunction in the hypothalamus, the temporal and frontal lobes. We report a 63 year-old man that developed neuropsychological disturbances with hypersexuality as a prominent feature, disinhibition and moderate memory loss, hypersomnia and irritability after a bilateral paramedian thalamic infarction. A SPECT showed frontal hypoperfusion. We believe that these findings are expression of frontal-subcortical circuits dysfunction, particularly the orbitofrontal circuit, secondary to dorso medial thalamic infarction which probably plays a role in the determination of human sexual behavior. This case favors a thalamic modulation of frontal function.

Study of oligoclonal bands restricted to the cerebrospinal fluid in multiple sclerosis patients in the city of São Paulo

The frequency of oligoclonal bands (OCB) restricted to the cerebrospinal fluid (CSF) from patients with multiple sclerosis (MS) varies widely in different populations. The objective of this study was to determine the frequency of these OCB in a group of MS patients in the city of São Paulo. Techniques used to detect OCB consisted of isoelectric focusing followed by immunoblotting. Oligoclonal bands were found in 49 (54.4%) out of 90 patients with clinically definite MS; in (31.2%) of the 16 patients with clinically isolated syndrome; in 7 (17.9%) of 39 patients with inflammatory disorders of the central nervous system (IDCNS), and in none of the individuals with no neurological condition (control group). The specificity of the method was 100% when compared to the control group and 82.1% when compared to the IDCNS group. These results suggest that the frequency of CSF OCB is much lower in Brazilian MS patients from São Paulo city than that reported in MS series from Caucasian populations.

Complete motor recovery after acute paraparesis caused by spontaneous spinal epidural hematoma: case report

BackgroundSpontaneous spinal epidural hematoma is a relatively rare but potentially disabling disease. Prompt timely surgical management may promote recovery even in severe cases.Case presentationWe report a 34-year-old man with a 2-hour history of sudden severe back pain, followed by weakness and numbness over the bilateral lower limbs, progressing to intense paraparesis and anesthesia. A spinal magnetic resonance imaging scan was performed and revealed an anterior epidural hematoma of the thoracic spine. He underwent an emergency decompression laminectomy of the thoracic spine and hematoma evacuation. Just after surgery, his lower extremity movements improved. After 1 week, there was no residual weakness and ambulation without assistance was resumed, with residual paresthesia on the plantar face of both feet. After 5 months, no residual symptoms persisted.ConclusionsThe diagnosis of spontaneous spinal epidural hematoma must be kept in mind in cases of sudden back pain with symptoms of spinal cord compression. Early recognition, accurate diagnosis and prompt surgical treatment may result in significant improvement even in severe cases.

Recommendations on diagnosis and treatment of depression in patients with multiple sclerosis

Multiple sclerosis (MS) is frequently associated with depression. Yet there are few clinical trials on treating depression in MS and no agreed recommendations for its assessment and follow-up. We present evidence-based recommendations for several aspects of depression in MS, including screening for depression, recognition of other concomitant psychiatric conditions, suicide risk, disability, fatigue, cognition, adherence to treatment, the effect of drugs used to treat MS on depression and possible pharmacological treatments for depression in MS.

Neuromyelitis optica with onset in childhood and adolescence.

BACKGROUND Neuromyelitis optica with onset before the age of 18 years is a relatively rare, yet potentially devastating condition. The objective of the present study was to contribute to the study of early-onset neuromyelitis optica with a case series. PATIENTS Data were collected from medical records of Brazilian neurologists caring for patients with neuromyelitis optica occurring in childhood and adolescence. RESULTS Twenty-nine patients with neuromyelitis optica occurring before the age of 18 years and fulfilling the diagnostic criteria were identified. The average age at disease onset was 13 years and the patients had had an average disease duration of 6 years. The expanded disability scale score at the latest consultation was, on average, 4.7, and one patient had died from the disease. The 29 patients had had an average 4.5 relapses during the disease, accounting for 0.75 relapses per year, irrespective of the medication used. All patients were using one or more of the following medications: azathioprine, prednisone, immunoglobulin, and glatiramer acetate. CONCLUSIONS Neuromyelitis optica with onset in childhood and adolescence is a poorly understood condition that is often disabling and difficult to manage.

Persistent Headache in Patients With Multiple Sclerosis Starting Treatment With Fingolimod

It is difficult to think of a medication that does not have “headache” as a potential adverse event listed by the manufacturer. However, this symptom is not described in detail by the pharmaceutical companies, and no considerations are made regarding the acceptable duration of this side effect or how to manage it, should it occur. This letter concerns 12 potential cases of headache associated with use of fingolimod, a new and very efficient drug for treating multiple sclerosis (MS). Upon starting with fingolimod, these patients presented persistent headache that resembled new daily persistent headache (NDPH) but were, in fact, attributable to a new substance. Fingolimod is an orally bioavailable compound that antagonizes the sphingosine 1-phosphate (SP1) receptor. In vivo, fingolimod is phosphorylated to fingolimod-phosphate, which resembles naturally occurring S1P. Through binding to SP1 sites in lymphocytes, fingolimod prevents these cells from leaving the lymph nodes. The first dose of fingolimod may be associated with bradycardia, fatigue, gastrointestinal disturbance, headache, and upper respiratory tract infection. Other adverse events in patients using fingolimod that are more serious but rare include atrioventricular block, herpetic viral infections, and macular edema. Although headache is described as a potential side effect of fingolimod and many other drugs for the treatment of MS, the present cases caught our attention because of the characteristics that these patients presented. These patients comprised 8 males and 3 females, of mean age 33.5 years (range 23 to 46 years), who are undergoing treatment for MS. Fingolimod was prescribed due to suboptimal response to other drugs. One patient had a previous history of episodic migraine. They were not using any drugs other than immunomodulatory treatments for MS. In all patients, their headache started within the first week of use of fingolimod and persisted for at least 3 months. In all cases, the pain was bilateral, non-pulsatile, of moderate intensity, and persistent From the MS Reference Center, Universidade Metropolitana de Santos, SP, Brazil (Y.D. Fragoso); MS Reference Center, Hospital Sirio-Libanes, Sao Paulo, SP, Brazil (T. Adoni); Department of Neurology, Hospital Beneficencia Portuguesa de Sao Paulo, Sao Paulo, SP, Brazil (S. Gomes); MS Unit, Centro Hospitalar Unimed, Joinville, SC, Brazil (M.V.M. Goncalves); Department of Neurology, Hospital Universitario Antônio Pedro, Universidade Federal Fluminense, Niterói, RJ, Brazil (A.P.C. Matta); Department of Neurology, Medical School, Irmandade de Misericordia da Santa Casa de Sao Paulo, Sao Paulo, SP, Brazil (M.F. Mendes); Department of Neurology, Universidade Regional de Blumenau, Blumenau, SC, Brazil (F. Siquineli).

Alternatives for reducing relapse rate when switching from natalizumab to fingolimod in multiple sclerosis

ABSTRACT Natalizumab is a therapeutic option for treating multiple sclerosis (MS) and is particularly efficacious for patients with highly active disease. A long washout period has been recommended between withdrawal of natalizumab and start of fingolimod (another option for treating MS). This long washout period has been associated with a significant increase in MS activity. In the present study, a group of 96 patients who were switched from natalizumab to fingolimod had short washout periods between drugs, or monthly corticosteroid pulse therapy if longer washout periods were recommended. This therapeutic approach led to the lowest reported relapse rate so far, among patients with MS switching from natalizumab to fingolimod (8.3%). No complications from short withdrawal were observed in this group of patients.

Cerebrum-cervical arterial dissection in adults during sports and recreation

UNLABELLED Dissection of cervical arteries constitutes a medical emergency. Although relatively rarely, activities classified as sports and recreation may be a cause of arterial dissection independently of neck or head trauma. The purpose of the present paper was to present a series of cases of cerebrum-cervical arterial dissection in individuals during or soon after the practice of these sports activities. METHODS Retrospective data on patients with arterial dissection related to sports and recreation. RESULTS Forty-one cases were identified. The most frequently affected vessel was the vertebral artery. A large variety of activities had a temporal relationship to arterial dissection, and jogging was the most frequent of these. This is the largest case series in the literature. CONCLUSION Arterial dissection may be a complication from practicing sports.