Denis Bernardi Bichuetti

Neuromyelitis optica in Brazil: a study on clinical and prognostic factors

Objectives To describe the clinical characteristics of patients with relapsing neuromyelitis optica (NMO) from a tertiary care center in Brazil and compare the groups with normal and abnormal brain magnetic resonance imaging (MRI). Methods Retrospective review of 41 patients followed at the Neuroimmunology Clinic of the Federal University of São Paulo, Brazil, from 1994 to 2007. Results All patients had relapsing-remitting optic-spinal disease, long extending spinal cord lesions, and brain MRI not meeting Barkhof criteria for multiple sclerosis (MS), thus fulfilling the 1999 and 2006 Wingerchuck criteria for NMO. Mean follow-up time was 52 months; mean age of onset was 32.6 years. The mean relapse rate (RR) and progression index (PI) were 1.0 and 0.9, respectively. Twenty-four patients had brain lesions not compatible with MS on MRI, and there were no statistical differences on PI and RR between patients who had brain lesions and patients who did not. Incomplete recovery, but not the type of first relapse, correlated with a worse prognosis. Seventeen patients were tested for NMO-IgG (anti-aquaporin-4 antibody) with 41% positivity. Conclusions In this series, we did not find a statistical difference of disease progression between patients with and without brain lesions, suggesting that the presence of brain abnormalities is not a marker of disease severity.

Lack of Response to Pulse Cyclophosphamide in Neuromyelitis Optica: Evaluation of 7 Patients

T he effect of immunosuppression with corticosteroids, azathioprine, mycophenolate, or rituximab for preventing relapses in patients with neuromyelitis optica (NMO) has been demonstrated in case series and retrospective studies. These drugs are currently considered the mainstay treatment for preventing neurologic worsening in NMO. Herein, we describe our experience with pulse cyclophosphamide for treating patients with NMO, which was used prior to azathioprine and had its use interrupted owing to lack of efficacy.

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.

White matter spectroscopy in neuromyelitis optica: a case control study.

BackgroundNaa/Cr ratio in normal appearing white matter (NAWM) of patients with multiple sclerosis (MS) is altered beyond plaques, suggesting early axonal loss, and correlates to clinical disability. Brain lesions not typical of MS have been described in Neuromyelitis optica (NMO), and correspond to brain aquaporin-4 channel sites, but the evaluation of Naa/Cr ratio in NAWM of patients with NMO and its association to the presence of brain lesions and clinical disability have not been described.ObjectivesTo evaluate the Naa/Cr of normal appearing white matter (NAWM) in 16 patients with NMO compared to healthy controls.MethodsWe performed brain magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS) of 16 patients with NMO and compared to age matched healthy controls.ResultsNAWM Naa/Cr did not show statistical difference among patients and controls, neither between patients that had normal brain MRI and atypical brain lesions.ConclusionNAWM was found to have a normal Naa/Cr in patients with NMO, reinforcing the concept that the white matter is not primarily affected in this disease.

Neuromyelitis optica: brain abnormalities in a Brazilian cohort

Neuromyelitis optica (NMO) is a demyelinating disease consisting of relapsing-remitting optic neuritis and myelitis with a more severe course than Multiple Sclerosis. Recently, it has been shown that almost 50% of patients with NMO can have brain magnetic resonance imaging (MRI) abnormalities. We report on six Brazilian patients with NMO, fulfilling the 1999 Wingerchuck criteria for this disease, with abnormal brain MRI and discuss their clinical and radiological features.

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.

Patients with neuromyelitis optica have a more severe disease than patients with relapsingremitting multiple sclerosis, including higher risk of dying of a demyelinating disease

UNLABELLED Although neuromyelitis optica (NMO) is known to be a more severe disease than relapsing-remitting multiple sclerosis (RRMS), few studies comparing both conditions in a single center have been done. METHODS Comparison of our previously published cohort of 41 NMO patients with 177 RRMS patients followed in the same center, from 1994 to 2007. RESULTS Mean age of onset was 32.6 for NMO and 30.2 for RRMS (p=0.2062) with mean disease duration of 7.4 years for NMO and 10.3 years for RRMS. Patients with NMO had a higher annualized relapse rate (1.0 versus 0.8, p=0.0013) and progression index (0.9 versus 0.6, p<0.0001), with more patients reaching expanded disability status scale (EDSS) 6.0 (39 versus 17%, p=0.0036). The odds ratio for reaching EDSS 6.0 and being deceased due to NMO in comparison to RRMS were, respectively, 3.14 and 12.15. CONCLUSION Patients with NMO have a more severe disease than patients with RRMS, including higher risk of dying of a demyelinating disease.

Bilateral SUNCT syndrome associated to chronic maxillary sinus disease

SUNCT syndrome (short lasting unilateral neuralgiform headache with conjuntival injection and tearing) is defined as short attacks of periorbital unilateral pain and accompanied by ipsilateral lacrimation and redness of the same eye. We present an unusual SUNCT case with bilateral pain that started five years ago after an acute maxillary sinus infection that evolved to chronic sinusitis. This association has been described in few SUNCT cases, but its causal role remains uncertain. The patient was a 58 years old man that fulfilled a headache diary that showed the usual circadian pattern, worsening in the morning and afternoon, and responded to treatment with gabapentina. He was submitted to a functional endoscopic sinus surgery and evolved with milder pain. In a review of 21 patients, 5 had a past medical history of sinusitis, but the causal role of this association remained uncertain.

Risk Acceptance in Multiple Sclerosis Patients on Natalizumab Treatment

Objective We aimed to investigate the ability of natalizumab (NTZ)-treated patients to assume treatment-associated risks and the factors involved in such risk acceptance. Methods From a total of 185 patients, 114 patients on NTZ as of July 2011 carried out a comprehensive survey. We obtained disease severity perception scores, personality traits’ scores, and risk-acceptance scores (RAS) so that higher RAS indicated higher risk acceptance. We recorded JC virus status (JCV+/-), prior immunosuppression, NTZ treatment duration, and clinical characteristics. NTZ patients were split into subgroups (A-E), depending on their individual PML risk. Some 22 MS patients on first-line drugs (DMD) acted as controls. Results No differences between treatment groups were observed in disease severity perception and personality traits. RAS were higher in NTZ than in DMD patients (p<0.01). Perception of the own disease as a more severe condition tended to predict higher RAS (p=0.07). Higher neuroticism scores predicted higher RAS in the NTZ group as a whole (p=0.04), and in high PML-risk subgroups (A-B) (p=0.02). In low PML-risk subgroups (C-E), higher RAS were associated with a JCV+ status (p=0.01). Neither disability scores nor pre-treatment relapse rate predicted RAS in either group. Conclusions Risk acceptance is a multifactorial phenomenon, which might be partly explained by an adaptive process, in light of the higher risk acceptance amongst NTZ-treated patients and, especially, amongst those who are JCV seropositive but still have low PML risk, but which seems also intimately related to personality traits.

Demyelinating disease in patients with myasthenia gravis

Myasthenia gravis (MG) is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.