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Milberto Scaff

Publications and source records attributed to Milberto Scaff.

At least 19 recordsLinked to original sources
PubMed2007

Bilateral occipital infarcts associated with carotid atherosclerosis and a persistent hypoglossal artery.

The persistent hypoglossal artery (PHA) is the second most common persistent embryological carotid-basilar connection and usually represents an incidental finding in cerebral arteriograms. The hypoglossal artery connects the primordial carotid artery with the longitudinal neural arteries, which later form the basilar artery. The PHA leaves the internal carotid artery as an extracranial branch, enters the skull through the anterior condyloid foramen, the hypoglossal canal and joins the caudal portion of the basilar artery. We report magnetic resonance and digital subtraction angiography findings in the first case of bilateral occipital infarctions associated with PHA and carotid atherosclerosis. The probable mechanism underlying bilateral occipital infarcts was embolism from the carotid territory to the posterior cerebral arteries. PHA may present a challenge in diagnosis and management of patients with carotid atherosclerosis and vertebrobasilar ischemia.

Aged↗
PubMed2006

Occurrence of nervous system involvement in SIRS.

Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age ranged from 16 to 95 years old. SIRS was caused by infection in 68.8% of patients, trauma in 10.9%, burns in 7.8%, and elective surgery in 4.7%. The central nervous system involvement occurred in 56.3% of patients and was characterized as encephalopathy in 75%, seizures in 13.9%, non-epileptic myoclonus in 2.8%, and ischemic stroke in 8.3%. The magnetic resonance imaging, cerebrospinal fluid and electroencephalographic changes were unremarkable in encephalopathic patients. Neuromuscular disorders were diagnosed in 43.7%. Critical ill polyneuropathy was characterized in 57.1%, critical ill myopathy in 32.1%, demyelinating neuropathy in 7.2%, and pure motor neuropathy in 3.6%. Nerve and muscle pathological studies dismissed inflammatory abnormalities. The identification of these conditions has important economic implications and may change the critically ill patients' prognosis.

Adolescent↗
PubMed2006

Wilson's disease with myoclonus and white matter lesions.

White matter lesions (WML) and epilepsy have been occasionally seen in Wilson's disease. No cases of generalized myoclonus have been reported so far. We present a patient with psychiatric symptoms starting at age 16, followed by tremor, generalized dystonia and severe generalized myoclonus. In addition to classical findings, the MRI showed also extensive WML in temporal, parietal and frontal regions, preserving interhemispheric fibers. Necropsy revealed marked alterations of white matter, cortex and basal ganglia. We subsequently review the literature concerning WML and myoclonus in Wilson's disease.

Adolescent↗
PubMed2006

Neurological manifestations in Wilson's disease: Report of 119 cases.

We describe the neurological manifestations of 119 patients with WD (93 index cases and 26 affected family members) seen between 1963 and 2004. The mean age at symptoms onset was 19.6 years (range, 7-37 years). Medical records were reviewed for the patient's first neurological examination. The most frequent neurological manifestations observed were dysarthria (91%), gait disturbance (75%), risus sardonicus (72%), dystonia (69%), rigidity (66%), tremor (60%), and dysphagia (50%). Less frequent manifestations were chorea (16%) and athetosis (14%). Rare neurological presentations were seizures (4.2%), and pyramidal signs (3%).

Adolescent↗
PubMed2006

Comparison between digital subtraction angiography and magnetic resonance angiography in investigation of nonlacunar ischemic stroke in young patients: preliminary results.

PURPOSE: We preliminarily investigated the relevance of performing digital subtraction angiography (DSA) in addition to magnetic resonance angiography (MRA) in definition of ischemic stroke etiology in young patients. METHOD: DSAs and MRAs from 17 young patients with nonlacunar ischemic stroke were blindly analyzed and their impact on stroke management was evaluated. RESULTS: Etiologies were the same considering results of either DSA or MRA in 12/17 cases. In 15/17 patients no changes would have been made in treatment, regardless of the modality of angiography considered. CONCLUSION: These preliminary results suggest that DSA may be redundant in two thirds of ischemic strokes in young patients. Further larger prospective studies are necessary to determine indications of DSA in this age group.

Adolescent↗
PubMed2006

Cerebral microbleeds and intravenous thrombolysis: case report.

Intravenous thrombolysis is an important procedure that has significant impact on ischemic stroke prognosis. However, intracranial hemorrhage (ICH) is a feared complication of this procedure. It has been suggested that cerebral microbleeds (CMBs) may increase the risk of ICH after thrombolysis. We report on a 69 years-old woman with multiple CMBs submitted to intravenous thrombolysis without complications.

Aged↗
PubMed2005

Facial sensory symptoms in medullary infarcts.

OBJECTIVE: To investigate the correlation between facial sensory abnormalities and lesional topography in eight patients with lateral medullary infarcts (LMIs). METHOD: We reviewed eight sequential cases of LMIs admitted to the Neurology Division of Hospital das Clínicas/São Paulo University between July, 2001 and August, 2002 except for one patient who had admitted in 1996 and was still followed in 2002. All patients were submitted to conventional brain MRI including axial T1-, T2-weighted and Fluid attenuated inversion-recovery (FLAIR) sequences. MRIs were evaluated blindly to clinical features to determine extension of the infarct to presumed topographies of the ventral trigeminothalamic (VTT), lateral spinothalamic, spinal trigeminal tracts and spinal trigeminal nucleus. RESULTS: Sensory symptoms or signs were ipsilateral to the bulbar infarct in 3 patients, contralateral in 4 and bilateral in 1. In all of our cases with exclusive contralateral facial sensory symptoms, infarcts had medial extensions that included the VTT topography. In cases with exclusive ipsilateral facial sensory abnormalities, infarcts affected lateral and posterior bulbar portions, with slight or no medial extension. The only patient who presented bilateral facial symptoms had an infarct that covered both medial and lateral, in addition to the posterior region of the medulla. CONCLUSION: Our results show a correlation between medial extension of LMIs and presence of contralateral facial sensory symptoms.

Adult↗
PubMed2005

Ullrich congenital muscular dystrophy and Bethlem myopathy: clinical and genetic heterogeneity.

UNLABELLED: Ullrich congenital muscular dystrophy (UCMD), due to mutations in the collagen VI genes, is an autosomal recessive form of CMD, commonly associated with distal joints hyperlaxity and severe course. A mild or moderate involvement can be occasionally observed. OBJECTIVE: To evaluate the clinical picture of CMD patients with Ullrich phenotype who presented decreased or absent collagen VI immunoreactivity on muscular biopsy. RESULTS: Among 60 patients with CMD, two had no expression of collagen V and their clinical involvement was essentially different: the first (3 years of follow-up) has mild motor difficulty; the second (8 years of follow-up) never acquired walking and depends on ventilatory support. A molecular study, performed by Pan et al. at the Thomas Jefferson University, demonstrated in the first a known mutation of Bethlem myopathy in COL6A1 and in the second the first dominantly acting mutation in UCMD and the first in COL6A1, previously associated only to Bethlem myopathy, with benign course and dominant inheritance. CONCLUSION: Bethlem myopathy should be considered in the differential diagnosis of UCMD, even in patients without fingers contractures; overlap between Ullrich and Bethlem phenotypes can be supposed.

Adolescent↗
PubMed2005

Dystrophin-glycoproteins associated in congenital muscular dystrophy: immunohistochemical analysis of 59 Brazilian cases.

UNLABELLED: The congenital muscular dystrophies (CMD) are heterogeneous muscular diseases with early and dystrophic pattern on muscle biopsy. Many different subtypes have been genetically identified and most phenotypes not yet identified belong to the merosin-positive (MP) CMD subgroup. OBJECTIVE: To analyze the immunohistochemical expression of the main proteins of the dystrophin-glycoproteins associated complex in muscle biopsy of patients with different CMD phenotypes, for investigating a possible correlation with clinical and histopathological data. METHOD: Fifty-nine patients with CMD had clinical, histopathological and immunohistochemical data evaluated: 32 had MP-CMD, 23 CMD with merosin deficiency (MD-CMD), one Ullrich phenotype and three Walker-Warburg disease. RESULTS: Dystrophin and dysferlin were normal in all; among the patients with MD-CMD, merosin deficiency was partial in nine who showed the same clinical severity as those with total deficiency; the reduced expression of alpha-sarcoglycan (SG) and alpha-dystroglycan (DG) showed statistically significant correlation with severe MD-CMD phenotype. CONCLUSION: There is a greater relationship between merosin and the former proteins; among MP-CMD patients, no remarkable immunohistochemical/phenotypical correlations were found, although the reduced expression of beta-DG had showed statistically significant correlation with severe phenotype and marked fibrosis on muscular biopsy.

Adolescent↗
PubMed2005

[Vestibular paroxysmia: clinical study and treatment of eight patients].

Vestibular paroxysmia is a syndrome of cross-compression of the VIII cranial nerve and was first described by Jannetta who used the term "disabling positional vertigo". This syndrome is characterized by brief attacks of vertigo, tinnitus, vestibular and auditory deficits. MRI may show the VIII nerve compression from vessels in the posterior fossa, such as the basilar, vertebral, anterior-inferior cerebellar or the posterior-inferior cerebellar arteries. Vestibular paroxysmia may be treated either with medical therapy, such as carbamazepine, phenytoin or gabapentin or with the microvascular decompression of the VIII nerve. This study describes eight patients with vestibular paroxysmia. Four of them showed also clinical signs suggesting cross-compression of the V and/or VII nerve. Seven patients treated with carbamazepine had significant improvement of vertigo and tinnitus.

Aged↗
PubMed2005

Status Dystonicus: study of five cases.

Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.

Adult↗
PubMed2005

[Diagnosis and treatment of the most frequent vestibular syndromes].

The aims of this study were to identify the most common vestibular syndromes in a dizziness unit, and to observe their clinical aspects and response to treatment. Five hundred and fifteen patients were studied retrospectively in two institutions. Aspects of anamnesis, physical examination and the response to treatment were evaluated. The most frequent syndromes were: benign paroxysmal positioning vertigo (VPPB) (28.5%), phobic postural vertigo (11.5%), central vertigo (10.1%), vestibular neuritis (9.7%), Meniere disease (8.5%), and migraine (6.4%). A good response to treatment was observed in most patients with migraine (78.8%), VPPB (64%), vestibular neuritis (62%), Meniere disease (54.5%) and vestibular paroxismia (54.5%). On the other hand, patients with downbeat nystagmus and bilateral vestibulopathy had poor response (52.6% and 42.8%, respectively). The diagnosis of these most frequent vestibular syndromes were established through anamnesis and physical examination (with specific clinical tests for evaluation of the vestibular function). The correct diagnosis and adequate treatment are important since these syndromes may have a good prognosis.

Female↗
PubMed2005

Ethnic differences in cerebral venous thrombosis.

BACKGROUND: Cerebral venous thromboses (CVT) with distinct clinical presentations have been shown worldwide. However, there is little information regarding race-ethnic differences in this disease. METHODS: We prospectively studied 50 CVT patients from Brazil, comparing clinical and laboratory data among white (W) and African-Brazilian (AB) patients. RESULTS: Seventy percent of the patients were female, 26 W and 23 AB, mean age 34.7 years. Multiple sinus CVT, deep CVT and worse outcome were significantly more frequent in AB than in W patients. There was a trend towards a higher frequency of factor V Leiden and prothrombin mutation in W than in AB, and of protein C deficiency in AB. CONCLUSIONS: CVT was more severe in AB patients than in W patients. Race-ethnic differences may account for the heterogeneous distribution of inherited thrombophilia in this series.

Adolescent↗
PubMed2005

Bilateral olivary hypertrophy after unilateral cerebellar infarction.

We describe a case of bilateral olivary hypertrophy and palatal tremor after unilateral cerebellar infarction. Hypertrophic olivary degeneration (HOD) is associated with hypersignal in the inferior olivary nucleus (ION), on T2-weighted images. HOD has been more often observed ipsilaterally to a central tegmentum tract lesion or contralaterally to a dentate nucleus or a superior cerebellar peduncle lesion. Double innervation of each ION from either dentate nucleus may have underlied the imaging and clinical findings in this 63 year-old male patient.

Cerebral Infarction↗
PubMed2005

Proton MR spectroscopy in Wilson disease: analysis of 36 cases.

BACKGROUND AND PURPOSE: Wilson disease (WD) is rare but one of the few metabolic disorders that can possibly benefit from effective available treatments. The literature regarding proton MR spectroscopy (MRS) in WD is scarce and controversial. The purpose of this study was to determine the brain metabolic changes due to WD by using MRS. To our knowledge, this is the first time that MRS was performed in such a large sample of patients with WD. METHODS: Thirty-six patients with WD and 37 healthy volunteers were examined with MRS in the parieto-occipital cortex, frontal white matter, and basal ganglia (BG). Ratios of the following metabolites were calculated in relation to creatine (Cr): N-acetylaspartate (NAA), choline (Cho), myo-inositol (mI), and glutamine/glutamate (Glx). The mean peak line width was measured on each spectrum. RESULTS: Compared with control subjects, patients with WD had significantly decreased NAA/Cr ratios in the three studied areas (P < .005) and an increased mI/Cr ratio in the BG (P < .001). Cho/Cr and Glx/Cr did not differ between the groups. The mean peak line in the BG was wider in patients than in control subjects. CONCLUSION: WD is unequivocally associated with MRS changes that could possibly be assigned to neuronal loss (in the three studied areas), to gliosis, and to iron and/or copper deposition in the BG.

Adolescent↗
PubMed2004

[The influence of ethanol in alcoholic beverages in extracranial carotid arteries atherosclerosis].

UNLABELLED: There is less incidence of occlusive cerebrovascular disease, of coronarian atherosclerosis and of other arteries with a light to moderate consumption of alcohol, suggesting that the same may occur with respect with the extracranial carotid arteries. Using color Doppler ultrasonography, we studied 328 extracranial carotid arteries of white male and females over 35 year old, with normal blood pressure, non-smokers and free of the main diseases make up the risk factors for cardiovascular diseases. They were stratified, according to the level of weekly alcohol consumption in milliliters (ml), in abstainers, light drinkers (1 to 100), moderate drinkers (101 to 300) and heavy drinkers (over 301). There was a lower incidence of atherosclerotic plaque and stenosis in the moderate drinkers. CONCLUSION: The study suggests that ethyl alcohol when drunk with moderation exerts a protective action from carotid atherosclerosis.

Adult↗