Terezinha de Jesus Teixeira Santos

Amyotrophic lateral sclerosis: clinical analysis of 78 cases from Fortaleza (Northeastern Brazil)

We report on the clinical characteristics of amyotrophic lateral sclerosis (ALS) in Fortaleza (Northeastern Brazil). For this, we analyzed retrospectively (from 1980 to 1999) 78 cases of ALS from the Service of Neurology of the University Hospital of Fortaleza diagnosed clinically and laboratorially (EMG, muscle biopsy, myelography, blood biochemistry, muscle enzymes and cranio-cervical X-ray). The results showed that they were mostly sporadic ALS (76/78), and they were divided into definite (n = 36), probable (n = 20), possible (n = 15) and suspected (n = 7), according to the level of diagnostic certainty. They were also subdivided into juvenile (n = 17), early-onset adult (n = 18), age-specific (n = 39) and late-onset (n = 4) groups. Clinically, they presented as initials symptoms, principally, asymmetrical (30/78) and symmetrical (24/78) weakness of extremities, besides bulbar signs, fasciculations, and atrophy. Curiously, pain as first symptom occurred in an expressive fashion (17/78). The predominant initial anatomic site, in this series, was the spinal cord, and mainly affecting the arms. As to the symptom accrual from region to region, this occurs more quickly in contiguous areas, and fasciculations are predominant when bulbar region was associated.

Pain in tropical spastic paraparesis/HTLV-I associated myelopathy patients

OBJECTIVE Tropical Spastic Paraparesis/HTLV-I Associated Myelopathy (TSP/HAM) is a chronic myelopathy, and pain has been mentioned as a frequent sensory symptom in this condition. The authors aimed at analyzing this symptom in a TSP/HAM patients series. METHOD For this, 46 patients were analyzed considering demographic and clinical characteristics and complaint of pain as to verbal description, time of onset and classification, correlated with the degree of motor disability and type of pain. RESULTS Among the 46 TSP/HAM patients, 28 (60.8%) complained of pain, predominant in the early phase of the disease. Most of the patients exhibited neuropathic characteristics of pain, correlated with increased motor disability. CONCLUSION Pain in TSP/HAM patients is a frequent and early symptom, and the neuropathic type is predominant (57.1%) and paralleled with increased incapacitation. The pathogenic involvement of cytokines may possibly be involved in the meaning of this symptom in this condition.

Western blot seroindeterminate individuals for human T-lymphotropic virus I/II (HTLV-I/II) in Fortaleza (Brazil): a serological and molecular diagnostic and epidemiological approach.

How to handle Western blot (WB) seroindeterminate individuals for Human T-lymphotropic Virus 1/2 (HTLV-1/2) constitutes a challenge for blood banks and families. We made a cross-sectional study of 191 enzyme linked immunoassay (EIA) reactive individuals from the hematological center (HEMOCE) of Fortaleza (Brazil), examining their serological (WB) and molecular (PCR) diagnosis, and demographic profiles, as well as a possible association of their condition with other infectious pathologies and risk factors. Ethical institutional approval and personal consent were obtained. Out of 191 EIA reactive individuals, 118 were WB seroindeterminate and 73 were seropositive for HTLV-1/2. In the PCR analysis of 41 WB seroindeterminate individuals, 9 (22%) were positive and 32 (78%) were negative for HTLV-1/2. The demographic analysis indicated a trend towards a predominance of males among the seroindeterminate individuals and females in the seropositive ones. The seroindeterminate individuals were younger than the seropositive ones. We did not find any association of these conditions with syphilis, Chagas disease or HIV or hepatitis, and with risk factors such as breast-feeding, blood transfusion, STD (syphilis) and IDU.

Neuropathic pain characteristics in patients from Curitiba (Brazil) with spinal cord injury

This was a descriptive cross-sectional study on patients with spinal cord injuries living in Curitiba, Paraná, Brazil. The aim was to evaluate the pain characteristics among such patients seen at referral care centers for spinal cord injury patients in Curitiba. A total of 109 adults with spinal cord injury in this city were evaluated regarding the presence of pain, especially neuropathic pain. Neuropathic pain was evaluated using the DN4 questionnaire, a universal instrument that has been translated and validated for Portuguese. A visual analog scale (VAS) was used to evaluate the intensity of pain. The prevalence of pain among these 109 patients was 31.2% (34 patients). The nociceptive pain presented was classified as musculoskeletal pain (nine patients), visceral pain (four patients) and mixed pain (one patient), thus totaling 14 patients (12.8%). Another 20 patients (18.3%) showed symptoms of neuropathic pain and fulfilled the criteria for neuropathic pain with scores greater than 4 out 10 in the DN4 questionnaire. Regarding the characteristics of the patients with neuropathic pain, most of them were male, younger than 40 years of age and paraplegic with incomplete lesions. They had become injured from 1 to more than 5 years earlier. The predominant etiology was gunshot wounds, and the intensity of their pain was high, with VAS scores greater than 5. This study partially corroborates other studies conducted on this subject. Studies of this type are important for understanding the profile of these patients, for the purpose of designing strategies for their rehabilitation, with a focus on the appropriate treatment and management of pain.

Motor neuron diseases in the University Hospital of Fortaleza (Northeastern Brazil): a clinico-demographic analysis of 87 cases

In this retrospective (1980-1998) study, we have analyzed clinico-demographically, from the records of the University Hospital of Fortaleza (Brazil), a group of 87 patients showing signs and symptoms of motor neuron diseases (MNDs). Their diagnosis was determined clinically and laboratorially. The WFN criteria were used for amyotrophic lateral sclerosis (ALS) diagnosis. The clinico-demographic analysis of the 87 cases of MNDs showed that 4 were diagnosed as spinal muscular atrophy (SMA), 5 cases as ALS subsets: 2 as progressive bulbar paralysis (PBP), 2 as progressive muscular atrophy (PMA) and 1 as monomelic amyotrophy (MA), and 78 cases of ALS. The latter comprised 51 males and 27 females, with a mean age of 42.02 years. They were sub-divided into 4 groups according to age: from 15 to 29 years (n= 17), 30 to 39 years (n= 18), 40 to 69 years (n= 39) and 70 to 78 years (n= 4). From the 78 ALS patients, 76 were of the classic sporadic form whilst only 2 were of the familial form. The analysis of the 87 patients with MNDs from the University Hospital of Fortaleza showed a predominance of ALS patients, with a high number of cases of juvenile and early onset adult sporadic ALS.

HTLV-I negative tropical spastic paraparesis: a scientific challenge

We reviewed the historical, clinical and etiological aspects of the progressive chronic spastic myelopathies of unknown etiology, disserting on the clinical similarities between HTLV-I seropositive and seronegative tropical spastic paraparesis (TSP), as well as focusing on the PCR studies of the seronegative TSP.

Neuropathology of two Brazilian autopsied cases of tropical spastic paraparesis / HTLV-I associated myelopathy (TSP/HAM) of long evolution.

We report on a neuropathological analysis of two cases of TSP/HAM originating from Brazil. These two cases had, respectively, an evolution of 13 and 40 years. The main neuropathological findings consisted of spinal cord atrophy, mainly the lower thoracic cord, diffuse degeneration of the white and grey matter, rare foci of mononuclear and perivascular cuffs, and hyaline hardening of arteriolae. The supraspinal structures were normal, excepting for a slight gliosis in the cerebellum. An analysis on the long evolutive cases as described in the literature is outlined in this study.

The effect of baclofen on spontaneous and evoked behavioural expression of experimental neuropathic chronic pain.

Baclofen (beta-p-chlorophenyl-GABA) has been used in humans to treat spasticity, as well as trigeminal neuralgia. Since GABA (gamma-aminobutyric acid) has been implicated in inhibitory and analgesic effects in the nervous system, it was of interest to study the effect of baclofen in experimental neuropathic pain. With this purpose, experiments were carried out in 17 neuropathic rats with constrictive sciatic injury, as described by Bennet and Xie (1988), taking as pain parameters scratching behaviour and the latency to the thermal nociceptive stimulus. The results showed that baclofen induces, in a dose-dependent manner, significant decrease (p < 0.05) of scratching behaviour and significant increase (p < 0.05) of the latency to the nociceptive thermal stimulus. The absence of antagonism of naloxone suggested a non-participation of an opioid-mediated mechanism in this analgesic effect of baclofen on experimental neuropathic pain.

Pharmacological, morphological and behavioral analysis of motor impairment in experimentally vitamin C deficient guinea pigs

The scurvy shows an inflammatory disease and gingival bleeding. Nevertheless, in an animal model for guinea pigs, described by Den Hartog Jager in 1985, scurvy was associated with a motor neuron disease with demyelinization of the pyramidal tract, provoking neurogenic atrophy of muscles. Aiming at searching the protective role of vitamin C in nervous system, a pharmacological, morphological and behavioral study was conducted. Three experimental groups were used: A100, animals receiving 100 mg/ vitamin C/ day; A5.0, animals receiving 5.0 mg/vitamin C/ day; and A0, animals without vitamin C. We analyzed the weight gain, muscular diameter and behavioral tests. In all tests examined, we found significant differences between the supplemented groups in comparison with scorbutic group (p<0.05). Thereafter, the animals were killed for histopathology of gastrocnemius muscle, spinal cord and tooth tissues. In addition, a morphometric study of periodontal thickness and alpha-motor neuron cell body diameter were done. The vitamin C-diet free regimen seemed to induce a disruption in spinal cord morphology, involving the lower motor neuron, as confirmed by a significant reduction in neuron perycaria diameter and muscular atrophy, complicated by increased nutritional deficit.

Polymyositis in childhood as clinical manifestation associated with HTLV-1

Laboratory of Experimental Neurology and Neurophysiology/DFF/UFC and Institute of Biomedicine of the Brazilian Semiarid/ INCT/CNPq: MD, MSc; MD, PhD, Full Professor of Neurology and Neurophysiology/DFF/UFC; MD, Medical Doctor of the Brasilia Base Hospital; MSc, PhD, Associate Professor of the Medicine Course of the Christus Faculty; DVM; DVM, PhD, Professor of the Superior School of Agriculture of Mossoro; DVM, PhD, Associate Professor of Histology/DM/UFC; MD, PhD, Associate Professor of Neuropsychology/DFF/UFC. The HTLV-1 (human T-cell lymphotropic virus type 1) is a retrovirus etiologically associated with tropical spastic paraparesis/HTLV-1 associated myelopathy (TSP/ HAM) and adult T-cell lymphoma/leukemia (ATLL). These two forms of clinical presentation are the best known and studied, although the virus may be also related with other clinical conditions, among them, the polymyositis. In Jamaica and Japan, the association between HTLV-1 seropositivity and polymyositis has been described in 85% and 16%, respectively. In the present study, we report a case of polymyositis associated with HTLV-1 as an uncommon form of clinical manifestation in a 10 years-old child mimicking muscular dystrophy. The possibility of vertical transmission (breast feeding) is outlined in this case. The parents of the patient signed a consent form.