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Biomedical subjects

Lineu C Werneck

Publications and source records attributed to Lineu C Werneck.

At least 19 recordsLinked to original sources
PubMed2006

Bone marrow transplantation in patients with storage diseases: a developing country experience.

Bone marrow transplantation (BMT) is a therapeutic option for patients with genetic storage diseases. Between 1979 and 2002, eight patients, four females and four males (1 to 13 years old) were submitted to this procedure in our center. Six patients had mucopolysaccharidosis (MPS I in 3; MPS III in one and MPS VI in 2), one had adrenoleukodystrophy (ALD) and one had Gaucher disease. Five patients had related and three unrelated BMT donor. Three patients developed graft versus host disease (two MPS I and one MPS VI) and died between 37 and 151 days after transplantation. Five patients survived 4 to 16 years after transplantation. Three patients improved (one MPS I; one MPS VI and the Gaucher disease patient), one patient had no disease progression (ALD) and in one patient this procedure did not change the natural course of the disease (MPS III).

Adolescent↗
PubMed2006

[Transcranial Doppler in the neurological practice].

Transcranial Doppler (TCD) was described in 1982 by Rune Aaslid and introduced in Brazil in 1992 by Roberto Hirsh in the University of São Paulo. Since the acquisition of TCD by Neurology Division of Hospital de Clínicas of Universidade Federal do Paraná (HC-UFPR) in 2001, the knowledge about TCD clinical utility has been increasing for diagnosis, follow-up and management of patients with cerebrovascular diseases. The objective of this study is to describe the current TCD indications and medical specialties indicators in our hospital. Although guidelines as published by the American Academy of Neurology establish values for TCD study with methods specificity and sensibility, we realize that the utility in our center depends on our efforts to show methods beneficial in various clinical settings.

Adolescent↗
PubMed2006

[Dropped head syndrome in motor neuron disease].

Dropped head is a syndrome caused by weakness of the neck extensor muscles found in different kinds of neuromuscular disorders and also in amyotrophic lateral sclerosis. This is a cases report of three women with motor neuron disease with beginning of dysphagia and cervical weakness that it evolved with dropped head. The investigation showed normal magnetic resonance imaging of brain and cervical column. Needle electromyography showed active and chronic denervation in bulbar muscles and cervical, thoracic and lumbosacral segments. We discuss the characteristic of disease, specially the clinical manifestations and electromyography features, with emphasis at the clinical evaluation of dropped head in the suspicion of motor neuron disease.

Aged↗
PubMed2006

Pure neural leprosy: diagnostic value of the polymerase chain reaction.

Pure neural leprosy (PNL) is often difficult to diagnose when acid-fast bacilli (AFB) cannot be detected. We undertook the present study to evaluate use of the polymerase chain reaction (PCR) in diagnosing PNL. Fifty-eight patients (41 men and 17 women) suspected of pure neural leprosy (PNL) were examined. Patients were classified as borderline tuberculoid (BT, 40 cases) and polar tuberculoid (TT, 18 cases) types. Nerve biopsy was performed and was positive for AFB in 20 patients (all BT patients), i.e., 34.5% of total cases. DNA was extracted from the nerve biopsy samples and amplified using PCR for a specific repeated sequence of DNA from Mycobacterium leprae. PCR analysis was positive in the nerve samples from 29 patients (50%), 27 of the BT type, and 2 of the TT type patients. Further, PCR analysis was positive in 14 of 38 cases that were negative for AFB by nerve biopsy, of which 12 were of the BT type and 2 the TT type. PCR analysis proved to be a useful method to investigate pure neural leprosy, enabling confirmation of the diagnosis in more than a third of the cases that were negative for AFB by nerve biopsy.

Adult↗
PubMed2006

Botulinum-A toxin in the treatment of painful post-stroke nocturnal paroxysmal dystonia triggered by periodic limb movements of sleep: case report.

INTRODUCTION: Sleep disorders presenting involuntary movements may be very annoying to patients, apart from their negative influence on sleep. OBJECTIVE: To report the use of botulinum type-A toxin (BoNT-A) to manage the case of a patient whose sleep was severely disrupted by episodes of dystonic posturing of the right lower limb triggered by periodic limb movements of sleep (PLMS). METHOD: A 79-year-old woman with mild post-stroke right hemiparesis presented with recurrent painful episodes of dystonia of the right lower limb, which disrupted her sleep. The dystonic episodes could also be voluntarily triggered by extension of the right hallux. Polysomnography confirmed that the dystonic episodes were triggered by PLMS. Twenty units of BoNT-A (20U/500U vial) were injected into her right extensor hallucis longus. RESULTS: Shortly after BoNT-A was injected, the dystonic symptoms abated, and the patient achieved better sleep efficiency. CONCLUSION: The PLMS-related involuntary extension of the hallux was probably triggering the nocturnal post-stroke lower limb dystonic paroxysms. BoNT-A injection into the right extensor hallucis longus was effective in managing this condition and thus resolved the associated disruption of sleep.

Aged↗
PubMed2005

Rosalie: the Brazilian female monkey of Charcot.

Jean-Martin Charcot, the father of Neurology, a very austere and reserved man that did not express affection freely for human being, had a profound affection to animals, particularly to a small female monkey, called "Rosalie", which came from Brazil and was a gift of Dom Pedro II to Charcot.

Animals↗
PubMed2005

Warfarin as a therapeutic option in the control of chronic cluster headache: a report of three cases.

Chronic cluster headache remains refractory to medical therapy in at least 30% of those who suffer from this condition. The lack of alternative medical therapies that are as effective as, or more effective than, lithium carbonate makes new therapies necessary for this highly disabling condition. Based on a previous report, we gave oral anticoagulants to three patients with chronic cluster headache. Two of them remained cluster headache-free while taking warfarin. In the third patient, the use of warfarin for three weeks initially increased the frequency and intensity of cluster headache attacks but subsequently induced a prolonged remission. In spite of the paucity of data available, oral anticoagulation appears to be a promising therapy for chronic cluster headache.

Administration, Oral↗
PubMed2005

Kennedy's disease phenotype with positive genetic study for Kugelberg-Welander's disease: case report.

We described a patient with clinical findings from Kennedy's disease and positive genetic study for Kugelberg-Welander's disease. A 24 years old man with negative family history presented with progressive spinal and bulbar muscular atrophy and gynecomastia at the age of 14. He was clinically diagnosed as having Kennedy's disease. However, a genetic study performed later was found to be negative for this disease and was positive for Kugelberg-Welander's disease, with deletion of the exons 7 and 8 in the "survival of motor neuron" gene.

Adult↗
PubMed2004

Cerebrotendinous xanthomatosis: report of two Brazilian brothers.

Cerebrotendinous xanthomatosis is a treatable rare autossomal recessive disease characterized by lipid storage secondary to a sterol 27-hydroxylase deficiency in the formation of cholic and chenodeoxycholic acids. We describe two Brazilian brothers with cognitive impairement and chronic diarrhea. One of them also presents bilateral cataracts. Neurological findings were progressive walking deficit, limb ataxia and pyramidal signs. Both patients had bilateral Achilles tendon xanthomata. Magnetic resonance image showed signal alterations in cerebellar hemispheres. We describe these cases with molecular genetic analysis confirming diagnosis and comparing with previous literature. The CYP27A1 gene study showed a C1187T mutation on exon 6.

Adult↗
PubMed2004

Hemorrhagic stroke after naphazoline exposition: case report.

Ten percent of all strokes are due to spontaneous cerebral hemorrhages. They are associated to drugs (licit and illicit) in 9.5% of all cases in young adults. This is a case report of a 44-year-old man, without previous morbidities, who presented a sudden onset headache and arterial hypertension 24 hours after use of naphazoline as nasal decongestant. Cranial tomography showed right thalamus hemorrhage. Cerebral angiography showed no aneurisms, vascular malformations or vasculitis. No other risk factors were found during investigation in this patient and the stroke was attributed to naphazoline exposition.

Adult↗
PubMed2004

Bizarre behavior during intracarotid sodium amytal testing (Wada test): are they predictable?

The intracarotid sodium amytal test (ISAT or Wada Test) is a commonly performed procedure in the evaluation of patients with clinically refractory epilepsy candidates to epilepsy surgery. Its goal is to promote selective and temporary interruption of hemispheric functioning, seeking to define language lateralization and risk for memory compromise following surgery. Behavioral modification is expected during the procedure. Even though it may last several minutes, in most cases it is subtle and easily manageable. We report a series of patients in whom those reactions were unusually bizarre, including agitation and aggression. Apart of the obvious technical difficulties (patients required physical restraining) those behaviors potentially promote testing delay or abortion and more importantly, inaccurate data. We reviewed those cases, seeking for features that might have predicted their occurrence. Overall, reactions are rare, seen in less than 5% of the ISAT procedures. The barbiturate effect, patients' psychiatric profiles, hemisphere dominance or selectiveness of the injection were not validated as predictors. Thorough explanation, repetition and simulation may be of help in lessening the risk of those reactions.

Adolescent↗
PubMed2004

Cerebellar hemorrhage as a complication of temporal lobectomy for refractory medial temporal epilepsy: report of three cases.

Cerebellar hemorrhage is listed among the potential complications following neurosurgical procedures. In this scenario it is usually reported as a rare condition. However, it seems that epilepsy surgery patients are somewhat more prone to this kind of complication, compared to other surgical groups. Head positioning, excessive cerebral spinal fluid draining and the excision of non-expanding encephalic tissue (or combinations among the three) are likely to be cause underlying remote cerebellar hemorrhage. Out of the 118 ATL/AH performed at our institution, between 1996 and 2002, we identified 3 (2.5%) patients presenting with cerebellar hemorrhage. We report on such cases and review the literature on the topic.

Adult↗
PubMed2004

Simvastatin-induced mononeuropathy multiplex: case report.

The association between the use of statins and neuromuscular disease is currently being intensely discussed. We relate a 63 years old man with possible case of statin-induced neuropathy in a patient with dislipidemia in use of simvastatina at high doses. The electrophysiologic studies disclosed findings compatible with mononeuropathy multiplex, suggested by clinical prescutation of asymmetrical numbness and weakness. More common causes of mononeuropathy multiplex were excluded and the patient improved after the discontinuation of the drug.

Humans↗
PubMed2004

Carotid transient ischemic attacks presenting as limb-shaking syndrome: report of two cases.

Limb shaking syndrome (LSS) is a rare presentation of transient ischemic attacks (TIAs), usually secondary to a critical carotid stenosis compromising intracranial circulation, first described 40 years ago. Two additional cases are described herein, aiming to add on to previous descriptions, and to warn physicians about this potentially harming and rather uncommon condition.

Aged↗
PubMed2004

[Clinical response to long action propranolol in 40 patients diagnosed with essential tremor with no previous treatment: an open, non-controlled study].

UNLABELLED: Essential tremor (ET) is the most common movement disorder and betablockers are still considered the first line of treatment. The aim of our study is to report the clinical response to long action propranolol (LAP) of 40 patients diagnosed with essential tremor with no previous treatment. METHOD: 40 patients with ET were evaluated with rating scales for severity of tremor and clinical classification of ET. All patients were evaluated at least twice, at enrollment and one month after starting treatment. RESULTS: thirty-six patients (90%) had type 2 ET and types 3 and 4 occurred in two patients each (10%). Familiar history was positive in 25 patients (62.5%). Mean age at first evaluation was 43.1 years and mean age at onset was 27.4 years. Of all patients, 33 (82.5%) had some degree of benefit and in 52.5 % this benefit was either good or excellent. CONCLUSION: LAP seems to be a good treatment option for ET in our series of 40 patients.

Adrenergic beta-Antagonists↗
PubMed2004

Myopathy of distal lower limbs: the clinical variant of Miyoshi.

Miyoshi distal dystrophy is a rare myopathy characterized by an autosomal recessive pattern of inheritance and it is prevalent in Japan. Onset of disease is in early adult life with weakness and atrophy of the leg muscles. Recently gene linkage to chromosome 2p12-14 has been established. We report three sisters, born of consanguineous parents. All of them noticed weakness and atrophy of leg muscles, and could not walk on their heels. In all of them the creatine kinase concentrations were very high. The electromyography showed myopathic patterns and the muscle biopsy disclosed dystrophic changes and an absence of dysferlin. There are few cases reported of Miyoshi distal dystrophy in Latin America. The Miyoshi myopathy may be distinct among the hereditary distal myopathies.

Adolescent↗
PubMed2004

Flunarizine and cinnarizine-induced parkinsonism: a historical and clinical analysis.

BACKGROUND: Drug-Induced Parkinsonism (DIP) represents the second leading cause of Parkinsonism (PK) in several countries. Flunarizine and cinnarizine are some of the most common drugs that cause DIP. This paper reviews the first description of Flunarizine and Cinnarizine-Induced Parkinsonism (FCIP), as well as the subsequent literature, emphasizing epidemiological, clinical and diagnostic aspects. METHODS: We reviewed the literature on the subject, with special emphasis on the first description and the later definition of the clinical syndrome that results from chronic use of flunarizine and cinnarizine. RESULTS: In 1984, De Melo-Souza reported the first description of flunarizine-induced PK in five patients. Other reports followed on FCIP, emphasizing the clinical features, which are symmetrical parkinsonism, and depression, affecting mainly elderly women. CONCLUSIONS: Eighteen years after the original description, FCIP is a recognized condition with specific clinical features, and is the second most common cause of parkinsonism in many countries.

Cinnarizine↗