Sérgio Augusto Pereira Novis

Electroneuromyography and neuromuscular findings in 16 primary hypothyroidism patients

There has been few reports on electroneuromyography (ENMG) changes in hypothyroidism. The objectives of the present study were to investigate the frequency of ENMG abnormalities in hypothyroidism and correlate them with neurological signs and symptoms and muscle enzyme levels; and to compare latency, amplitude and nerve conduction velocity from selected nerves with controls. Sixteen patients suffering from primary hypothyroidism were submitted to ENMG before treatment. ENMG abnormalities were found in 87.5% of the patients; 46.6% had myopathy and 43.7% had carpal tunnel syndrome. There was no case of polyneuropathy. A clear-cut clinical, laboratorial and ENMG correlation was observed in patients with myopathy and carpal tunnel syndrome. The patients showed a significant tendency of nerve conduction slowness as compared with controls. The findings are in accordance with the well-known nerve and muscle damage in hypothyroidism.

Diagnosis of HAM/TSP based on CSF proviral HTLV-I DNA and HTLV-I antibody index

The contribution of human T-cell lymphotropic virus (HTLV-I) DNA by PCR in CSF and the intrathecal synthesis of antibodies to HTLV-I by the antibody index (AI) to the diagnosis of HTLV-1-associated myelopathy (HAM)/tropical spastic paraparesis (TSP) were evaluated. Cases of spastic paraparesis compatible with HAM/TSP had increased AI for HTLV-I (60/73) and HTLV-I proviral sequences in CSF (25/27). Among 27 patients with other neurologic diseases, three had increased AI and another three had positive HTLV-I DNA in CSF. Thus, the combination of PCR for proviral DNA and AI for HTLV-I in CSF provides consistent criteria for the diagnosis of HAM/TSP.

The value of csf analysis for the differential diagnosis of HTLV-I associated myelopathy and multiple sclerosis

Cerebrospinal fluid (CSF) and serum of 17 patients with HAM/TSP (HTLV-I associated myelopathy/tropical spastic paraparesis), six with multiple sclerosis and six with idiopathic epilepsy (non inflammatory control) from Brazil were analysed for the presence of intrathecal synthesis of virus-specific antibodies against measles, rubella, varicella zoster virus and herpes simplex virus by enzyme-linked immunosorbent assay (ELISA). All HAM/TSP and multiple sclerosis cases had an intrathecal immune response (oligoclonal IgG). In HAM/TSP, only 1/17 case showed a polyspecific intrathecal immune response against measles and rubella virus. In multiple sclerosis, specific antibodies against measles and rubella (MRZ response) were observed in all patients but not in the control with idiopathic epilepsy. The diagnostic and theoretical relevance of mono- and polyspecific immune responses is discussed for these chronic neurological diseases.

Spinal cord compression due to extramedullary hematopoiesis in the proliferative phase of polycythemia vera

Extramedullary hematopoiesis is a common accompaniment of a variety of hematologic diseases such as hereditary spherocytosis, thalassemia and myelofibrosis. The association of extramedullary hematopoiesis with polycythemia vera in the proliferative phase is much less usual. We report a patient who presented with paraplegia due to spinal cord compression; clinical investigation revealed a paravertebral hematopoietic tumor, and the diagnosis of polycythemia vera was then established.

Experience with t-PA treatment in a large south American City

BACKGROUND AND PURPOSE Early thrombolysis with intravenous recombinant tissue plasminogen activator (t-PA) reduces disability following ischemic stroke. However, only a small fraction of the potential candidates receive therapy. We studied the proportion of eligible patients who did not receive t-PA and the reasons therapy was withheld from patients admitted to a Brazilian university hospital. METHODS From July 1996 to June 1997, each suspected case of hyperacute ischemic stroke led to the immediate evaluation by a research physician. Patients first seen in primary care centers near the research hospital were to be rapidly transferred. Reasons for patient exclusion were detailed. RESULTS Fifty-six patients were evaluated; only 5 received t-PA therapy. The main reason for not receiving t-PA (34 cases) was late hospital arrival. This was caused by lack of recognition of the early stroke signs (n=13); delayed personal or family response (n=7); wrong medical orientation (n=4); and late patient transportation (n=10). A computed tomographic (CT) scand could not be done fast enough to allow therapy in 8 patients. Nine of 14 patients in whom neurological evaluation and CT scan were done within the 3-hour period were also excluded. The main reason was refusal of informed consent (n=5). Only a few patients were excluded because of medical contraindications (n=3) or the presence of early mass effect (n=1). CONCLUSIONS The main factor limiting access of patients with ischemic stroke to t-PA therapy in Rio de Janeiro is a low public awareness of early stroke symptoms and of the need for urgent hospital evaluation. Public education, an efficient transport system for suspected stroke patients, and optimization of the use of available CT scanners could have a significant impact on the number of treated patients.

Involuntary movements and AIDS: report of seven cases and review of the literature

We studied 1086 AIDS patients in the last six years. Of these 389 (35.82%) had neurological manifestation and 7 (1.8%) male patients had abnormal involuntary movements (parkinsonism in 3, hemichorea-hemiballism in 2, spinal myoclonus in 1 and rubral tremor in another). All patients were men, 5 white and 2 black. Four were homosexual, 2 drug-users and 1 bisexual. The mean age was 33.14 years. The time between AIDS diagnosis and the onset of movement disorders was 23.8 months in 5 patients and in 2 it was the first symptom. The parkinsonian patients did not show any opportunistic infection in connection with the neurological symptoms but in the remaining four cases this relationship was suggested. The data showed that not only the opportunistic infection but also the AIDS virus may play an important role on the development of involuntary movements.

Paroxysmal dystonia and neuromyelitis optica

Paroxysmal dyskinesias (PD) are thought to be rare movement disorders. The overwhelming majority of reported cases are primary. Secondary PD has seen reported to occur in some conditions, mainly in multiple sclerosis and head trauma. The anatomic origin of the lesion is also rarely seen at the spinal cord. Our objective was to describe four patients with paroxysmal dystonia secondary to spinal lesions during the recovering phase of a neuromyelitis optica (NMO) bout. In the reviewed literature, we do not find any report of PD related to NMO.

Determinação volumetrica do infarto cerebral na fase aguda usando tomografia computadorizada de crânio sem contraste: estudo comparando três métodos

The determination of lesion volumes in acute cerebral Infarction (CI) has important prognostic and therapeutic implications. Three different methods -computerised technique, linear planimetry and the A.B.C/2 formula - were compared in 27 patients using non-contrast computerised tomography (CT) made at hospital admission (first 48 hours following symptom onset). Two independent observers performed the measurements. The Intra-class correlation coefficients (ICCs) were determined. The three methods exhibited high interrelation. There was a close correlation between the linear planimetry as well as the ABC/2 formula and the computerised method with ICCs of 0.94-0.95 and 0.835-0.90 respectively. Linear planimetry and the ABC/2 formula were also highly correlated, with ICCs between 0.97 and 0.99. In conclusion, the computerised method is probably the most accurate. Linear planimetry and the ABC/2 formula are also reliable, however. The A.B.C/2 formula should be applied routinely to CI volume determination based on its low cost and fast calculation.

Holmes tremor in an HIV positive patient worsened by immune recovery inflammatory syndrome (IRIS).

IRIS is characterized by a paradoxical deterioration of clinical status after initiation of Anti-Retroviral Therapy (ART), despite improved immune function. It is caused by inflammatory response against the infectious antigen [1]. IRIS typically occurs in patients with a low initial CD4 (usually <50) and a rapid decline in viral load [2]. It is seen within a broad spectrum of HIV-related opportunistic infectious diseases and autoimmune disorders in patients who had been given Highly Active Anti-Retroviral Therapy (HAART) [3]. Our objective is to describe an HIV positive patient with Holmes ́ tremor worsened by IRIS, with marked recovery after therapy with steroids.

Multiple sclerosis outcome and morbi-mortality of a Brazilian cohort patients

We studied the clinical and evolution characteristics of multiple sclerosis (MS) patients followed since the onset of HUCFF/UFRJ in 1978. The diagnosis of MS was based on Posers et al. and MC Donalds et al. criteria. From 188 patients, 122 were included. Eighty-five were females. The mean age onset was 32.2 years-old (range 6.0 to 61.0+/-10.3), mainly Caucasians (82/67%). The relapsing-remitting course (MSRR) was more frequent (106/86.8%). Monosymptomatic onset was significantly more frequent in Caucasians than in Afro-Brazilians (p<0.05). Seventeen patients had benign form of MS and these patients presented association with MSRR when compared with severe form (p=0.01). The mortality rate was 2.12% (4 patients died). This study was similar to other Brazilian series with regard to sex and age, and lack of correlation between EDSS and number of relapses; it confirmed south-southeast African-descendants gradient distribution and association between first mono-symptomatic relapses and Caucasian; we found lower frequency of benign forms.