Recently, patients with hypohidrotic/anhidrotic ectodermal dysplasia (H/AED) have been reported to have a higher prevalence of symptoms suggestive of atopic disorders than the general population. To better understand atopic diathesis in H/AED, 6 cases of clinically or genetically diagnosed H/AED were examined. The following criteria were evaluated with patient consent: sweating, blood test resu...

PURPOSE Although different types of headache have been described in Chiari malformation type I, reports of cluster-like headaches are rare. CASE REPORT We report a 26-year-old man who presented with a two-week history of excruciating headache in the right temporal region after coughing, which was accompanied by autonomic features including right-sided nasal congestion and tearing from his rig...

Ectodermal dysplasias are rare conditions with a triad of hypotrichosis, anodontia and anhidrosis. In literature review there have been only a few reports of anesthetic management of patients with ectodermal dysplasias. Hyperthermia is a very serious risk which may occur due to the defect of sweat glands. The present case involves a 10-year-old child with ectodermal dysplasia who presented with...

A six-year-old girl with congenital sensory neuropathy with anhidrosis (CSNA) presented with bilateral hip dysplasia and subluxation on the right side. Conservative treatment of the hips by closed reduction and a plaster cast was unsuccessful. When aged seven years the patient had an intertrochanteric varus rotation osteotomy on the right side, but subluxation was again evident after five month...

ably affects development, as well as maintenance of neurons, because there is neuropathological and clinical progression. Inherited autonomic neuropathies are a rare group of disorders associated with sensory dysfunction. As a group they are termed the "hereditary sensory and autonomic neuropathies" (HSAN). Classification of the various autonomic and sensory disorders is ongoing. Phenotypic exp...

Objective To explore the aetiology of congenital insensitivity to pain with anhidrosis (CIPA) in two Chinese siblings with typical CIPA symptoms including insensitivity to pain, inability to sweat, and self-mutilating behaviours. Methods Clinical examination and genetic testing were conducted of all available family members, and the findings were used to create a pedigree. Mutation screening us...

BACKGROUND Heatstroke is a life-threatening condition defined by failure of heat load dissipation, resulting in a core temperature higher than 40 °C (104 °F) associated with neurological dysfunction. Topiramate may cause anhidrosis, potentially resulting in heatstroke, as reported especially in children. CASE PRESENTATION A 57-year-old Caucasian man was admitted to the emergency room in a feb...

We sought to identify the appropriate exercise intensity for unbiased comparisons of changes in rectal temperature (ΔTre) and local sweat rates (LSR) between groups unmatched for body size during uncompensable heat stress. Sixteen males vastly different in body morphology were separated into two equal groups [small (SM): 65.8 ± 6.2 kg, 1.8 ± 0.1 m2; large (LG): 100.0 ± 13.1 kg, 2.3 ± 0.1 m2], b...

Autonomic dysregulation is part of the complex degenerative process in amyotrophic lateral sclerosis (ALS). To investigate this, sweating was examined at rest in 39 patients with ALS in comparison with a control group. Sweat was collected over a 30 second period over the thenar and hypothenar eminences and on the sole of the foot, using a commercial device based on vapour pressure gradient. The...

Neurophysiology. Nerve growth factor (NGF) is a neurotropic factor essential for the survival and maintenance of NGF-dependent neurons. NGF-dependent neurons include NGF-dependent primary afferents and sympathetic postganglionic neurons in the peripheral nervous system (PNS). NGF-dependent neurons in the PNS form an interface between the nervous system and the body-proper (the organism minus th...