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Biomedical subjects

A Pou

Publications and source records attributed to A Pou.

At least 19 recordsLinked to original sources
PubMed1999

Laryngeal framework surgery for the management of aspiration.

BACKGROUND: During the past decade, laryngeal framework surgery has become the treatment of choice for the management of adductor paralysis of the vocal fold. The primary impetus for the use of this technique has been on the rehabilitation of voice. The purpose of this study was to ascertain the effectiveness of laryngeal framework surgery, including medialization laryngoplasty with silicone (MLS), with or without arytenoid adduction (AA), on eliminating aspiration, improving diet, and aiding in the subsequent decannulation of individuals with glottic insufficiency secondary to vocal fold palsy. METHODS: A retrospective chart review was performed on all patients initially seen with vocal cord paralysis who were treated with laryngeal framework surgery from June 1992 to April 1996. The study comprised 70 patients, including 31 women and 39 men, with a median age of 57 years. Clinical information was obtained regarding the etiology of the lesion, characteristics of the vocal cord deficit, history of aspiration, the presence of other neurologic deficits or concurrent pulmonary disease, treatment, and outcome. To determine the effectiveness of MLS, with or without AA, we assessed the final outcome regarding the presence and degree of aspiration, diet, history of aspiration pneumonia, and decannulation. RESULTS: Seventy patients underwent 77 MLS (three bilateral, four revisions), and 21 AA. Decreased aspiration was obtained in 96% of our patients. Seventy-five percent of those patients who had required a tracheotomy were decannulated. CONCLUSIONS: These results support the use of laryngeal framework surgery for the effective treatment of aspiration in selected patients initially seen with deficits of the glottic closure secondary to vocal fold paralysis or paresis.

Adult↗
PubMed1998

The anterior inferior cerebellar artery infarcts: a clinical-magnetic resonance imaging study.

Acute infarcts of the anterior inferior cerebellar artery (AICA) are unusual. We report 15 cases of AICA infarcts and their correlation with the topography of the lesion by brain MRI. During 2 years we prospectively identified 7 cases of AICA infarcts among 770 acute strokes (0.9% of the acute strokes seen in our department). We studied these cases and also another 8 that we found retrospectively. Most patients (8/15) had a unilateral affectation of both middle cerebellar peduncle (MCP) and inferior lateral pontine area (ILP), in these cases the main symptoms were vertigo, ataxia, peripheral facial palsy and hypoacusia. Two other patients had isolated MCP infarcts and were characterized by peripheral vertigo and ataxia, without hypoacusia or facial palsy. Another 2 patients had isolated ILP territory infarct characterized by vertigo, left peripheral facial palsy without hypoacusia and mild or no ataxia. One patient had a Gasperini syndrome. Finally 3 patients had bilateral AICA infarcts due to basilar thrombosis. The etiology was atherosclerosis in 9 patients, lacunar due to hypertension in 1, cardiac embolism in 1, migraine in 1 and unknown in 3. Among the 15 patients only 2 died, both with AICA plus infarcts. In the remaining patients a follow-up during a mean of 31 months (3 months to 12 years) showed no recurrences.

Adult↗
PubMed1998

Multiple endocrine involvement in two pediatric patients with Kearns-Sayre syndrome.

We present 2 cases of progressively severe Kearns-Sayre syndrome (KSS) with multisystemic affectation and atypical endocrine and cutaneous features, a 16-year-old patient (case 1) and a 5-year-old patient (case 2). Endocrine studies showed high glucose and glycohemoglobin concentrations with normal pancreatic reserve and low values of ACTH, cortisol, LH and FSH in case 1. Normal ACTH values with low concentrations of cortisol and PTH were observed in case 2. Southern blot analysis and PCR amplification revealed the presence of a deletion of approximately 6.7 kb in the mitochondrial DNA of both patients. Endocrinological studies suggest that adrenal insufficiency may be an additional feature of KSS that worsens the clinical evolution of the patients. In spite of a normal pancreatic reserve, insulin therapy should be considered in patients with diabetes mellitus of mitochondrial origin.

Adolescent↗
PubMed1998

[Oral anticoagulation in the secondary prevention of cerebrovascular disease. Long-term follow-up of 169 patients].

INTRODUCTION: Although the indications for oral anticoagulation (AO) in the treatment of cerebral vascular disease (CVD) are well established, their potential side effects continue to give cause for worry. OBJECTIVES: To describe the complications and ischemic relapses in patients treated with AO for secondary prevention of CVD of cardiac embolic origin. PATIENTS AND METHODS: We included 169 patients with embologenic cardiopathy who, following an CVD, were treated with AO and followed-up at our medical centre for at least three months. We recorded their past clinical history and risk factors, occurrence of vascular relapses (VR), complications involving hemorrhage (CH), and data regarding course and follow-up. RESULTS: During an average follow-up of 50.3 months of a total of 707.9 patient/years, 20 VR (2.8% per year) were recorded; 15 of these were cerebro-vascular and mainly mild. We recorded 59 CH in 41 patients (8.3% per year) of which 6 were considered to be major. There was a 30% drop-out rate from follow-up at our centre, mainly due to death from other causes or to change of referral centre. CONCLUSIONS: There is a low incidence of relapse and of complications (usually mild) following AO for the secondary prevention of CVD of cardio-embolic origin. Efficacy and security are maintained in the long term.

Acenocoumarol↗
PubMed1996

Tracheotomy in children with juvenile-onset recurrent respiratory papillomatosis: the Children's Hospital of Pittsburgh experience.

Despite the risk of airway obstruction, tracheotomy has been viewed with trepidation in the management of recurrent respiratory papillomatosis (RRP). The literature suggests that the injury associated with the tracheotomy site may initiate the progression of disease to the distal airway. Alternatively, patients who require tracheotomy for RRP may be predisposed to distal spread because of more aggressive disease. In an effort to clarify this issue, we reviewed the Children's Hospital of Pittsburgh experience with 35 patients with RRP between 1984 and 1994; 13 patients received tracheotomies. Tracheotomy patients presented at a younger age with more widespread disease, often involving the distal airway prior to tracheotomy. Although distal spread occurred in 50% of patients, it was generally limited to the tracheotomy site. Overall, outcome in the tracheotomy group was satisfactory. Complications related to the tracheotomy were rare. We conclude that tracheotomy is an appropriate option for significantly airway compromise in patients with RRP.

Child↗
PubMed1996

[Motor neuron diseases. Present].

Recent progress in our understanding of motor neuron diseases, particularly those of degenerative pathogenesis such as spinal muscular atrophies (SMA) and amyotrophic lateral sclerosis (ALS), have to be described as historic. They are essentially of three types: 1) In first place are advances afforded by molecular genetics both in SMA (with the discovery of survival motor neurons and neuronal apoptosis inhibitor protein, which are markers of the disease and their pathogenetic mechanism) and in ALS (with the discovery of the super oxide dismutase [SOD1] gene, involved in the genesis of familial forms of ALS and other types of SMA such as certain forms of familial juvenile ALS, bulbar-spinal atrophy with gynecomasty and others). 2) In second place are biological data detailing the mechanisms of neuron death, whether programed or not, and emphasizing the importance of the so-called trophic or neurotrophin factors-whether nerve growth factor, brain-derived-neurotrophic factor, neurotrophin-3 or others whose effect of preventing neuron death has been demonstrated in vitro-as well as that of other substances such as Ca(2+)-activated neutral protease, which stabilizes synapses during development. It is assumed that one or another of these data will lead to therapeutic strategies for blocking the cascade of events that lead to neuron degeneration. 3) Finally, the strong impact of neuroimmunology in the field of neuromuscular pathology has been of interest mainly in neurogenic diseases marked purely and essentially be motor expression. As markers and pathogens, antiganglioside antibodies must necessarily be determined at this time in such entities as multifocal motor neuropathy, Guillain-Barré syndrome, acute axon motor neuropathy, Miller-Fisher syndrome and others, as their presence can inform therapeutic approaches. These three aspects and others currently under discussion will be treated in this course. At the same time the basic diagnostic aspects of motor neuron diseases will be emphasized: electrophysiologic assessment, on the one hand, and clinical features on the other. Establishing an exhaustive classification of SMA, from the earliest forms of infancy to adult types, is of great priority, as is exposing the full range of SMA according to whether distal or proximal predominance of atrophy is present. Our current understanding of the field is summarized in ten chapters on degenerative motor neuron diseases.

Amyotrophic Lateral Sclerosis↗
PubMed1996

[Juvenile and adult forms of spinal muscular atrophies].

We propose a classification system for spinal muscular atrophies (SMA) based on the distribution of clinical signs, paresis and atrophy, as well as on the location of the responsible gene and the resulting enzyme deficiency, whenever these are known. This highly practical classification system encompasses three large SMA groups, as follows. A) Generalized forms, many of which are hereditary, are generally transmitted in a recessive autosomal manner. The course of disease is more severe when symptoms manifest early. Patients whose symptoms first occur after the first year of life often reach adolescence and even adulthood, confirming a highly apparent congruence of intermediate and pseudomyopathic juvenile forms. The same genetic defect, deletion in the 5q11-13.3 locus, that is responsible for acute infantile SMA has been demonstrated in both the aforementioned forms. B) Focal forms are restricted and often isolated cases; when they are hereditary, the genetic profile is highly heterogeneous. Though the disease will not necessarily evolve, it may progress to a generalized form. Focal forms may be symetric, assymetric, spinal-bulbar or multisegmental. The genetic abnormality has been identified for only some forms, such as chronic bulbar-spinal amyotrophy linked to the X-chromosome, at whose location, Xq11, the androgenic receptor is found. C) Amyotrophic lateral sclerosis (ALS) manifests clinically in a variety of ways and may be isolated, familial, juvenile or associated. Familial ALS is related to a gene defect in the 21q22.1 location that codifies for the superoxide dismutase enzyme. One juvenile form of ALS is related to a defect in the 2q33-35 chromosome. Any type of SMA can be related to degenerative neuronal disease of the central nervous system, especially juvenile ALS with generalized SMA, although such a link is at present merely an attractive hypothesis. Specific bibliographic references are given for each SMA form. Figures are provided to illustrate most of the SMA forms included in this classification system, the patients being at this time older adolescents and adults whose disease has been in evidence over many years.

Adolescent↗
PubMed1995

Hereditary spastic paraparesis with dementia, amyotrophy and peripheral neuropathy. A neuropathological study.

Hereditary, probably autosomal recessive, spastic paraparesis in two siblings was associated with dementia of frontal lobe type, amyotrophy and peripheral sensory and motor polyneuropathy. Neuropathological findings correlate with neurological deficits, although neuron loss in the caudate and putamen, substantia nigra, and loss of Purkinje cells were clinically silent. Loss of neurons occurred in all cortical layers of the prefrontal lobe and superior temporal gyrus. Immunohistochemical studies showed reduced parvalbumin immunoreactivity in dendrites, and reduced numbers of calbindin D28k-immunoreactive cells, thus suggesting involvement of cortical local-circuit neurons. Myelin loss, ubiquitin-immunoreactive granular deposits, and nerve fibre degeneration in the white matter of the frontal lobes and corpus callosum were also observed. Cerebral and subcortical white matter abnormalities, together with atrophy of the thalamic dorsomedial complex and anterior nucleus, may have accounted for the development of severe dementia in this patient.

Brain↗
PubMed1995

Motion of the cerebellar tonsils in Chiari type I malformation studied by cine phase-contrast MRI.

We studied the effects on CSF dynamics at the foramen magnum and the clinical significance of the abnormal tonsillar motion in 14 patients with Chiari type I malformation and 14 control subjects using cine phase-contrast MRI. Dynamic MRI consisted of axial and sagittal cine phase-contrast sequences. CSF and tonsillar motion were qualitatively and quantitatively evaluated, and the subarachnoid space at the foramen magnum measured. In Chiari patients, cine phase-contrast MRI detected the abnormal pulsatile motion of the cerebellar tonsils, which produced a selective obstruction of CSF flow from the cranial cavity to the spine. The amplitude of the tonsillar pulsation and the severity of the arachnoid space reduction were associated with the symptom of cough-strain headache, but not with the presence of syringomyelia. The finding of abnormal valve dynamics of the cerebellar hernia revealed by cine phase-contrast MRI conforms to the pathophysiologic mechanisms suggested in pressure register studies and opens a new possibility in the presurgical assessment of Chiari patients with exertional symptoms.

Adult↗
PubMed1992

[Neurologic syndromes associated with anti-Hu antibody. Study of 24 patients].

BACKGROUND: Twenty-four patients with neurologic involvement and anti-Hu antibodies were studied with the aim of defining the type of tumor associated, evaluating whether the clinico-pathologic picture agreed with the concept of paraneoplastic encephalomyelitis (PEM) and evaluating the treatments used. METHODS: The study was retrospective with the clinical histories being reviewed to define the neurologic syndromes, their evolution and response to the different treatments, time of appearance and type of tumor as well as the neuropathologic changes in the patients undergoing autopsy. RESULTS: In 18 patients a neoplasm was diagnosed as small cell pulmonary carcinoma (SCPC) in 89% of the cases. The neurologic picture preceded the tumor by an average of five months. The clinical pictures included: sensitive neuropathy (20 patients), cerebellous and truncus encephalicus involvement (8 patients), motor neuropathy (6 patients), cortical involvement (5 patients) and neurovegetative dysfunction (4 patients). In 55% of the patients more than one area was altered. Post mortem studies carried out on 5 patients demonstrated inflammatory infiltrates and neuronal loss in multiple areas of the nervous system. None of the patients improved with treatment. The 9 patients who only received immunodepressants evolved in a way similar to those who were not treated. In 7 of the 11 patients who received antitumoral therapy, the neurologic syndrome stabilized for at least 6 months. CONCLUSIONS: The clinicopathological picture and the associated tumor seen in patients with anti-Hu antibodies are identical to those seen in PEM. Antitumoral treatment seems to be more effective than immunodepressant treatment.

Adult↗
PubMed1991

Surface area-independent assessment of lung microvascular permeability with an amphipathic tracer.

A combination of an amphipathic-indicator-dilution (ID) diffusing tracer 1,4[14C]butanediol (B) and a hydrophilic tracer ([14C]urea) (U) was hypothesized to provide a capillary surface area- (S) independent assessment of lung microvascular permeability (P). We performed ID studies on isolated perfused dog lungs and administered randomly two interventions, increasing P by alloxan infusion and reduction in S by lobar ligation. The ratio of PS product of U (PSU) to that for butanediol (PSB) was sensitive to changes in P yet insensitive to changes in S. We performed ID studies in which the dependence of PSU and PSB on flow, hematocrit, and plasma protein binding were examined. Measurements of PSU and PSB after flow and hematocrit were changed suggested that these factors have no significant independent effects. From ID and in vitro studies we also found that no significant binding of B to plasma proteins (albumin) occurred. We concluded that ID techniques using B and U provide a consistent measure of P, despite changes in S, hematocrit, plasma protein concentration, and recruitment.

Animals↗
PubMed1990

Normal proprioceptive trigeminal afferents in patients with Sjögren's syndrome and sensory neuronopathy.

A pure sensory neuropathy due to neuronal damage in the gasserian and spinal ganglia has been described in patients with Sjögren's syndrome. Conventional electromyographic (EMG) studies can demonstrate the selective involvement of the sensory pathways but they do not provide definite evidence for the site of the lesion. Noting that the trigeminal sensory neurons carrying cutaneous and muscular afferents are differentially located in the gasserian and mesencephalic nuclei, respectively, we carried out an electrophysiological study of the trigeminofacial and trigeminotrigeminal reflexes in 5 patients with Sjögren's syndrome and pure sensory neuropathy, in 10 patients with sensory-motor neuropathies of other causes, and in 10 healthy subjects. Our results show that patients with Sjögren's syndrome and pure sensory neuropathy who exhibited abnormal blink reflexes and an abnormal, cutaneous-induced masseter silent period had normal jaw jerks, whereas patients with sensory-motor neuropathies who exhibited abnormal cutaneous responses had abnormal jaw jerks. These findings suggest that the lesion in pure sensory neuropathy involves damage to the neurons of the gasserian ganglia and not to the trigeminal axons, since an axonal lesion would be expected to involve the large axons from muscle spindle receptors.

Adult↗
PubMed1990

Pure sensory neuropathy in patients with primary Sjögren's syndrome: clinical, immunological, and electromyographic findings.

A pure sensory neuropathy caused by lymphocytic infiltration of the dorsal root ganglia has been reported in a few patients with Sjögren's syndrome. The clinical, immunological, and electromyographic findings of five patients with this type of neuropathy and primary Sjögren's syndrome were reviewed. Typical clinical indications were the presence of a chronic asymmetrical sensory deficit, initial disease in the hands with a predominant loss of the vibratory and joint position senses, and an association with Adie's pupil syndrome or trigeminal sensory neuropathy. The simultaneous impairment of the central and peripheral evoked cortical potentials suggested that there was a lesion of the neuronal cell body. The neuropathy preceded the diagnosis of Sjögren's syndrome in four patients. Four patients were positive for Ro antibodies, but systemic vasculitis or malignancy was not found after a mean follow up of six years. These findings indicate that in patients with a sensory neuropathy the diagnosis of Sjögren's syndrome has to be considered, even if the patient denies the presence of sicca symptoms, and that appropriate tests must be carried out.

Adie Syndrome↗