OBJECTIVES The purpose of this study was to investigate the effect of a mandibular advancement device on different grades of obstructive sleep apnea using a relatively simple test for the apnea-hypopnea index to determine if a mandibular device will be effective. MATERIAL AND METHODS A total of 68 patients with obstructive sleep apnea syndrome (OSAS) including, 31 with mild, 23 with moderate ...

OBJECTIVE The aim of this paper was to report the first case of Kennedy disease misdiagnosed as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. CLINICAL PRESENTATION AND INTERVENTION A 58-year-old Chinese man presented with limb numbness, progressive limb proximal weakness, lymph node and thyroid enlargement, edema, pigmentation in the l...

UNLABELLED Adams Oliver syndrome (AOS) is a highly variable entity with terminal transverse limb defects (TTLD) and aplasia cutis congenita (ACC) with a wide phenotypic spectrum. Several inheritance models have been observed; the most severe phenotype has been related to an autosomal recessive (AR) pattern of inheritance. OBJECTIVE. To present a family with two half siblings with a severe pheno...

Multiple muscle agonists and antagonists acting for all the joint movements in the upper and lower limb exist. This redundancy of motor control is very valuable in normal physiology, but when a central nervous system injury with resulting upper motor neuron syndrome takes place, the source of the functional impairment may be difficult to localize. In this paper we discuss the use of gait and mo...

Sweet's syndrome is a neutrophilic dermatosis with worldwide distribution that has been associated with inflammatory autoimmune diseases, infections, malignancies, drugs, and pregnancy. The disease is idiopathic in up to 50% of patients. A 64-year-old woman, diagnosed with right limb cellulitis (4 days of evolution), was seen at our department, due to persistent cellulitis and progressive appea...

Goldenhar syndrome is a sporadic or inherited genetic syndrome characterized by limbal dermoids, preauricular skin tags and mandibular hypoplasia. Vertebral abnormalities may occur. The incidence of this syndrome ranges from 1 in 5,600 to 1 in >20,000 live births. It consists of abnormalities involving the first and second branchial arches. The etiology of the syndrome is heterogeneous. Diagnos...

Guillain-Barré syndrome is clinically characterized by acute onset of generalized, symmetrical, and ascending muscle weakness and areflexia from peripheral nerve involvement. In Guillain-Barré syndrome variants, however, some patients have unusual distribution of muscle involvement. Pharyngeal-cervical-brachial variant of Guillain-Barré syndrome is characterized by oropharyngeal, neck, and uppe...

Servelle Martorelle Syndrome is a congenital vascular malformation associated with soft tissue hypertrophy and bony hypoplasia. This rarely involves whole of an extremity, with involvement of part of limbs reported in literature. We present a case of a twelve year boy who presented to the Department of Plastic Surgery SGPGIMS in April 2011 ,with history of circumferential soft tissue hypertroph...

INTRODUCTION Crouzon syndrome is an autosomal dominant genetic disease characterized by bicoronal craniosynostosis, exorbitism with hypertelorism, and maxillary hypoplasia with mandibular prognathism. CASE OUTLINE We present the first reported case of Crouzon syndrome associated with a bilateral con- genital cholesteatoma of the temporal bone and discuss about the potential pathogenesis. CO...