Surgical and Camouflage Orthodontic Treatment of Anterior Open Bite: Comparison of Profile Attractiveness.

OBJECTIVE: To evaluate the profile's attractiveness between the different protocols for treating the anterior open bite. METHODS: The sample comprised 39 patients with anterior open bite treated with or without extractions, divided into two groups: The surgical group (G1) comprised 21 subjects (10 males, 11 females) with a mean initial age of 21.86 years (SD = 5.09), treated with fixed orthodontic appliance followed by orthognathic surgery, for a total mean period of 2.53 years (SD = 0.61). The mean overbite was -5.01 mm (SD = 2.50); The camouflaged group (G2) comprised 18 subjects (9 males, 9 females), with a mean initial age of 20.47 years (SD = 4.19), treated only with fixed orthodontic appliance, for a total mean period of 2.56 years (SD = 0.94). The mean overbite was -4.28 mm (SD = 1.15). Lateral photographs from pretreatment and posttreatment were used. These photographs were evaluated by 46 laypeople and 67 dentists, who rated the attractiveness of each profile from 0 (most unattractive profile) to 10 (most attractive profile). Intergroup comparisons were performed with independent t-tests. RESULTS: Both groups presented improvement in the profile attractiveness with treatment (p < 0.001). Before treatment, the profile of the surgical group was significantly less attractive than the profile of the camouflaged group (p < 0.001). The surgical presented a more attractive profile at the final stage than the camouflaged group (p < 0.001). The surgical group showed a greater improvement in profile attractiveness with treatment than the camouflaged group (p < 0.001). CONCLUSION: In the final stage, the surgical presented a more attractive and greater improvement in profile attractiveness than the camouflaged group. The laypeople and dentists judged similarly the initial profile attractiveness.

PLP1 duplication at the breakpoint regions of an apparently balanced t(X;22) translocation causes Pelizaeus-Merzbacher disease in a girl.

PLP1 (proteolipid protein1 gene) mutations cause Pelizaeus-Merzbacher disease (PMD), characterized by hypomyelination of the central nervous system, and affecting almost exclusively males. We report on a girl with classical PMD who carries an apparently balanced translocation t(X;22)(q22;q13). By applying array-based comparative genomic hybridization (a-CGH), we detected duplications at 22q13 and Xq22, encompassing 487-546 kb and 543-611 kb, respectively. The additional copies were mapped by fluorescent in situ hybridization to the breakpoint regions, on the derivative X chromosome (22q13 duplicated segment) and on the derivative 22 chromosome (Xq22 duplicated segment). One of the 14 duplicated X-chromosome genes was PLP1.The normal X chromosome was the inactive one in the majority of peripheral blood leukocytes, a pattern of inactivation that makes cells functionally balanced for the translocated segments. However, a copy of the PLP1 gene on the derivative chromosome 22, in addition to those on the X and der(X) chromosomes, resulted in two active copies of the gene, irrespective of the X-inactivation pattern, thus causing PMD. This t(X;22) is the first constitutional human apparently balanced translocation with duplications from both involved chromosomes detected at the breakpoint regions.

Cephalometric evaluation of Class II malocclusion treatment with cervical headgear and mandibular fixed appliances.

The present study aimed to evaluate the cephalometric changes in Class II patients treated exclusively with cervical headgear (CHG) in the maxillary arch and fixed appliances in the mandibular arch as compared with a control group. The sample comprised 82 lateral cephalograms obtained pre- (T1) and post- (T2) treatment/observation of 41 subjects, divided into two groups: group 1-25 Class II division 1 patients (20 females and five males), with a mean pre-treatment age of 10.4 years, treated for a mean period of 2.5 years and group 2-16 Class II untreated subjects (12 females and four males), with a mean initial age of 9.9 years, followed for a mean period of 2.2 years. Treatment changes between the groups were compared by means of t-tests. The results showed restriction of maxillary forward displacement and also a restriction in maxillary length growth, improvement in the maxillomandibular relationship, restriction of mandibular incisor vertical development, reduction in overjet and overbite, and improvement in molar relationship. It was concluded that this treatment protocol corrected the Class II malocclusion characteristics primarily through maxillary forward growth restriction.

Dengue infection: neurological manifestations and cerebrospinal fluid (CSF) analysis.

Neurological manifestation is considered a rare complication of dengue infection. Neurological and cerebrospinal fluid (CSF) findings of 13 patients with dengue infection were studied. Seven patients had encephalitis, two had myelitis and four showed Guillain-Barré syndrome (GBS). No alteration in CSF was found from 57% of those with encephalitis. Patients with GBS and myelitis showed a CSF-blood barrier dysfunction. The differences in the CSF may be related to the location of the lesion and multiple mechanisms of the disease in the nervous system.

Dioclein, a new nitric oxide- and endothelium-dependent vasodilator flavonoid.

In the present work, the vasorelaxant effect of dioclein, a new flavonoid isolated from Dioclea grandiflora (Leguminoseae), was investigated in the rat aorta. Dioclein induced a concentration-dependent relaxation in vessels pre-contracted with phenylephrine (IC(50)=1.3+/-0.3 microM), a response which was abolished after endothelium removal. Neither indomethacin (10 microM), an inhibitor of cyclo-oxygenase, nor atropine (1 microM), an antagonist of muscarinic receptors, modified the effect of dioclein. Dioclein (30 microM) induced a significant increase in guanosine 3':5'-cyclic monophosphate (cyclic GMP) levels in aortic rings with endothelium. The nitric oxide (NO) synthase inhibitor, N(G)-nitro-L-arginine-methyl-ester (L-NAME, 300 microM), strongly inhibited or abolished the relaxing effect and rise in cyclic GMP levels induced by dioclein. Furthermore, dioclein (30 microM) had no effect on the endothelium-independent relaxation produced by the NO donor, 3-morpholino-sydnonimine (SIN-1), while superoxide dismutase (100 U ml(-1)) significantly potentiated it. These results indicate that, in the rat aorta, dioclein induces a NO- and endothelium-dependent vasorelaxant effect, which is associated with cyclic GMP elevation. This vasorelaxation likely results from enhanced synthesis of NO rather than enhanced biological activity of NO.

Tapia's syndrome caused by Paracoccidioidis brasiliensis.

Tapia's syndrome is due to extracranial involvement of the XIIth nerve and the recurrent laryngeal branch of the Xth nerve. There is ipsilateral paralysis of vocal cords, soft palate and tongue. The main causes are parotid and other tumors or injuries to the high neck. We describe here a Brazilian female patient who presented with a lesion in the nasal mucosa, and soon after had dysphagia and dysphonia. Examination revealed paralysis of the soft palate, vocal cords and tongue ipsilaterally. Microscopic examination of the lesion in the nasal mucosa revealed the presence of the fungus. The patient was treated with sulfonamide and ketoconasol.

Modification of Ca2+ metabolism in the rabbit aorta as a mechanism of spasmolytic action of warifteine, a bisbenzylisoquinoline alkaloid isolated from the leaves of Cissampelos sympodialis Eichl. (Menispermaceae).

The regulation of intracellular Ca2+ as a mechanism of spasmolytic activity of a bisbenzylisoquinoline alkaloid, warifteine, isolated from the leaves of Cissampelos sympodialis, Eichl (Menispermaceae) was studied in the rabbit aorta. Warifteine (pD2' 4.12 +/- 0.09) similar to verapamil (pD2' 6.89 +/- 0.05) antagonized, in a noncompetitive and reversible manner, KCl-induced contractions, mediated by Ca2+ entry through voltage-operated channels. Noradrenaline-induced sustained contractions mediated by Ca2+ entry through receptor-operated channels were also inhibited by warifteine (IC50 6.03 x 10(-5) M) and the standard agent sodium nitroprusside (IC50 1.9 x 10(-8) M). In Ca(2+)-free medium, the alkaloid reduced the intracellular Ca(2+)-dependent transient contraction to noradrenaline by inhibiting the release of Ca2+ (IC50 2.6 x 10(-5) M) from the stores and the refilling (IC50 1.9 x 10(-5) M) of the intracellular stores. The standard agent, procaine, also inhibited the release of Ca2+ (IC50 3.2 x 10(-5) M) but had no significant effect on Ca2+ uptake into the stores. Warifteine failed to affect intracellular Ca2+ stores sensitive to caffeine, while procaine inhibited (IC50 7.9 x 10(-4) M) the release of Ca2+ from these stores. The results indicate that warifteine may cause muscle relaxation by inhibiting Ca2+ channels and by modifying the intracellular Ca2+ stores sensitive to noradrenaline.

[Axonal polyneuropathy in Chagas disease]. / Polyneuropathie axonale dans la maladie de Chagas.

We report the case of a 44-year-old woman presenting with chronic symmetrical, sensitive polyneuropathy on the lower limbs in the course of Chagas' disease. The electrophysiological findings were in keeping with axonal degeneration. The histological data displayed axonal degeneration with perivascular inflammatory mononuclear cells in the epineurium, with some neutrophils and eosinophils. Mononuclear cells surrounding endoneurial vessels were observed. Laboratory data did not suggest neither a mixed connective tissue disease nor a collagen vascular disorder. Hematological disease, malignancies, drug-or medicine-induced neuropathy were ruled out. The polyneuropathy in this case was probably related to Trypanosoma cruzi infection on account of the presence of high levels of anti-T. cruzi antibodies, and an immune mechanism might play a role in the vasculitic process.

[Cytomegalovirus encephalo-myelo-radiculitis in acquired immunodeficiency syndrome]. / Encéphalo-myélo-radiculite à cytomégalovirus au cours du syndrome d'immuno-déficience acquise.

A 30 year-old male, with the acquired immune deficiency syndrome (AIDS) presented with rapidly progressive flaccid paraplegia and sphincter incontinence. Cerebrospinal fluid examination showed elevated protein and pleocytosis. Death occurred 2 months after the onset of neurological signs. Post-mortem examination showed inflammatory necrotic lesions, relatively sparing the axons and predominantly involving the roots of the cord. Numerous cytomegalovirus (CMV) inclusion bodies were found in the necrotic lesions, in the subarachnoid spaces and in Schwann cells. CMV encephalitis and involvement of the 3rd cranial nerves were also observed. Only 8 well-documented clinico-pathological cases of acute CMV myeloradiculitis, which all presented as progressive cauda equina syndrome, have been reported until now in AIDS patients.

Non-progressive juvenile spinal muscular atrophy of the distal upper limb (Hirayama's disease): a clinical variant of the benign monomelic amyotrophy.

Hirayama's disease (HD) is frequently found in Asia, and is rarely referred among westerners. It affects young people with higher incidence in males. It is a focal distal amyotrophy with unilateral or asymmetric bilateral involvement of C7, C8 and T1 innervated muscles. HD appears sporadically and has a benign evolution with clinical stabilization in around one year. We report four young male patients with clinical and electrophysiological alterations described in HD, which were followed-up during 5 years. Electromyographic findings were indicative of lower motor neuron involvement. We analyzed cervical MRI aiming at understanding if a questionable spinal cord compression could be implicated in the pathogenesis, but no abnormality was verified. In view of its clinical, and EMG characteristics, HD is no more than a benign monomelic amyotrophy (BMA) clinical variant, and not a specific disease. This eponym could be considered only for the distal upper limb variant (Hirayama's variant) of the BMA.

[Clinico-pathologic correlations in 78 biopsies of the sural nerve]. / Correlação clínico-patológica em 78 biópsias de nervo sural.

Peripheral nerve biopsies when processed with conventional techniques for paraffin embedding usually do not provide sufficient data for the diagnostic conclusion. However, if the nerve is processed for resin embedding for semi and ultra-thin sections and teasing of fibres, several aspects can be analysed including quantitative and morphometric data. We studied the sural nerve biopsy of 78 patients examined at the Antonio Pedro University Hospital, Niterói RJ, applying those techniques and we found that in 55 cases (70.5%) the pathologic diagnosis was conclusive, in 11 (14.1%) although the nerve had abnormalities it was not possible to establish a diagnosis, and in 12 (15.4%) the nerve was normal. In 68 cases there was a clinical diagnosis which was confirmed in 49 but not in the remaining 19, since 8 had non-specific changes and 11 were normal. From the 10 cases which did not have a clinical diagnosis the biopsy was conclusive in 6, showed non-specific changes in 4, and was normal in 1 case. The pathologic conclusion in most of our cases was possible because not only we had the clinical data but all the nerves were processed for resin embedding.

[Descending ocular myopathy]. / Miopatia ocular descendente

The case of a 23 years old female patient, with primary involvement of the extraocular and faringeal muscles without familiar history is reported. Electromyographic and muscular biopsy studies proved the myogenic nature of the process. A clinical comparison between the ocular myopathy and the descending ocular myopathy is made, the authors thinking that both of them would be variants of the same muscle disease.

[Cortico-striate spinal degeneration. Report of a case with pathological and clinical findings suggestive of Creutzfeldt-Jakob disease]. / Degeneração cortico-estriato-medular. Relato de um caso com achados clinicos e patologicos sugestivos de doença de Creutzfeldt-Jakob

A case suggestive of Creutzfeldt-Jakob disease in an adult with widespread lesions of the cerebral cortex, striatum and the spinal cord is reported. Anatomopathological studies showed neuronal degeneration, astrocytic proliferation, intense lymphoplasmocytic activity and slight spongiform degeneration. This inflammatory lymphocytic infiltration corroborates the hypothesis of viral etiology of this disease.

Benign monomelic amyotrophy: a study of twenty-one cases.

A consecutive series of 21 patients with single limb atrophy (monomelic amyotrophy) is reported. Sixteen had lower limb atrophy and five had upper limb involvement. The median age of the onset was 20 years. Characteristic features were sporadic occurrence, wasting confined to one limb, insidious onset with slow progression, stabilizing in 1 to 4 years, and absence of pyramidal signs. All the patients with upper limb involvement were male, however in our cases with lower limb amyotrophy there were no male preponderance. We observed wasting of the entire length of the lower limbs in six patients. There were nine cases with amyotrophy restricted to the leg and one with amyotrophy only in the thigh. In the upper limb in four cases the involvement was distal and in one patient the atrophy was proximal. The electromyographic features were suggestive of anterior horn disease not only in the affected limb but also, in some cases, in clinically uninvolved limb. Cervical or lumbar MRI was normal. MRI of the lower limb disclosed increased signal intensity in the gastrocnemius and soleus muscles in one patient suggesting denervation.

[Hereditary proximal neurogenic muscular atrophy (Wohlfart-Kugelberg-Welander disease). Electromyographic, anatomo-pathologic and clinical study in 3 brothers]. / Atrofia muscular proximal neurogenica hereditaria (doença de Wohlfart-Kugelberg-Welander) Estudo clinico, eletromiografico e anatomo-patologico de 3 irmãos

The cases of three brothers with proximal weakness and muscles atrophies beginning in childhood are reported. Muscles biopsies and electromyographic studies have shown neurogenic pattern of atrophy and a dystrophy-like picture. It is concluded that this histological and eletromyographic picture can occur in pure partial denervation of long standing. The histopathologic study of the spinal cord of one patient revealed degenerative changes and loss of anterior horn ganglion cells. This is the fourth case of Wohlfar-Kugelberg-Welander disease with postmortem examination.

[Post-anoxic hemi-myoclonic encephalopathy: report of a case]. / Encefalopatia hemi-mioclônica pós anóxica: registro de um caso.

A case of a young man with myoclonus in his right side after cerebral hypoxia is reported. This patient had cerebral hypoxia caused by injury in his left common carotid artery. After a few hours he had generalized convulsive seizures of tonic-clonic type and also a septic shock. As consciousness was regained, he developed action and intention myoclonus in his right-side. The EEG showed diffuse typical myoclonus potentials. Clonazepan 8 mg daily was used with good results. This is the first reference in the medical literature of the unilateral localization in the Lance-Adams syndrome. The authors think that in this case two abnormalities contributed to the cerebral anoxia: the common carotid artery injury and the septic shock.

[Polyneuropathy caused by ethylene oxide. Report of a case with clinical, electrophysiological and histopathological studies]. / Polineuropatia devida ao óxido de etileno. Registro de caso com estudo clinico, eletrofisiológico e histopatológico.

A man who worked as an operator in a factory of sterilization of heat-sensitive materials has been exposed to ethylene oxide for seven years. He developed a mild sensori-motor polyneuropathy. The electromyography and nerve condition studies showed an axonal degenerative type of neuropathy. The sural nerve biopsy revealed mild loss of myelinated fibers, some fibers with axonal degeneration, some clusters of regeneration and few rows of myelin ovoids in the teased nerve fiber preparation. This is the first report of ethylene-oxide polyneuropathy in Brazil.

Guillain-Barré syndrome and head trauma. Case report.

The authors report the case of a 29 years old male patient presenting classical Guillain-Barré syndrome following head trauma. Only one other similar case is described in the literature. Head trauma as a precipitating event of the disease is discussed.

Chronic recurrent Guillain-Barré syndrome: report of 3 cases.

The classical Guillain-Barré syndrome is an acute or subacute polyradiculo-neuropathy whose main clinical features are progressive weakness of the limbs, decrease or absence of tendon reflexes, and sensory changes. Although in most of the cases there is complete recovery in weeks or months, some patients have a slow and progressive relapsing course and present thickening of the peripheral nerves. In this paper we describe three cases of the chronic and relapsing variety of Guillain-Barré syndrome, two of which had prominent hypertrophic changes in the peripheral nerves with onion bulb formations. The clinical and pathological features of this disease are reviewed. The three patients improved with the use of steroids.

[Association of myopathies and alcoholic cardiomyopathy: clinical, electroneuromyographic and histopathologic study of the skeletal muscle in 10 cases of alcoholic cardiomyopathy]. / Associação de miopatia e miocardiopatia alcoólicas: Estudo clínico, eletroneuromiográfico e histopatológico do músculo esquelético em 10 casos de miocardiopatia alcoólica.

Myocardial and skeletal muscle impairment caused by alcohol has been thoroughly studied. Nevertheless, the simultaneous involvement of those tissues by ethanol has not been broached in medical literature. We have studied ten patients undergoing alcoholic cardiomyopathy. They were subjected to a detailed neurological examination, muscle enzymes serum level determinations, electromyography, and muscle biopsy with analysis of the tissue by usual histological techniques and by electron microscopy. Only one of ten patients exhibited proximal weakness and atrophy of the lower limbs, the electromyographic and histological findings correlating with the clinical feature of the chronic muscle disease due to alcohol consumption. The electromyographic findings included muscle disease in eight patients, neuron disease in one patient and no changes at all in the tenth patient. In one patient only, the skeletal muscle proved normal when examined by conventional histological techniques. In the other nine patients there were several minimal changes, such as the proliferation of nuclei beneath the sarcolemma, atrophy of fibers, hyalinization, vacuolation, loss of muscle fiber striae, and atrophy of groups of fibers. Electron microscopy demonstrated the following changes in nine patients: intracellular edema, dissociation of myofilaments, alteration of the Z line, changing in the shape and increase in the number of mitochondria, thickening of sarcolemma, and vacuolation and increase of the glycogen granules. Since all patients exhibited skeletal muscle injury, we concluded that there is a close relation between alcoholic cardiomyopathy and skeletal muscle disease.