Fukuyama congenital muscular dystrophy (FCMD) is a rare, autosomal recessive form of muscular dystrophy (weakness and breakdown of muscular tissue) mainly described in Japan but also identified in Turkish and Ashkenazi Jewish patients; fifteen cases were first described on 1960 by Dr. Yukio Fukuyama.FCMD mainly affects the brain, eyes, and muscles, in particular, the disorder affects development of the skeletal muscles leading to weakness and deformed appearances, and brain development is blunted affecting cognitive functioning as well as social skills. In 1995, the disorder was linked to mutations in a gene coding for the protein fukutin (the FCMD gene). Fukuyama congenital muscular dystrophy is the second most prevalent form of muscular dystrophy in Japan. One out of every 90 people in Japan is a heterozygous carrier. Symptoms and signs
In terms of the signs/symptoms of Fukuyama congenital muscular dystrophy it is characterized by a decrease in skeletal muscle tone as well as an impairment in brain and eye development. Initial symptoms of FCMD present in early infancy as decreased ability to feed. Marked differences in facial appearance occur due to decreased muscle tone. Further characteristics include:
Seizures
Delay in development
Cardiac issues
Swallowing difficulty
Neurological problemsFukuyama congenital muscular dystrophy also affects the nervous system and various associated parts. FCMD affects normal development of the brain producing a broadly smooth, bumpy shaped cortex named cobblestone lissencephaly as well as various other malformations, notably micropolygyria. Children also experience delayed myelination in the brain.
Also advised are measures to avoid moisture build-up including keeping the groin region dry, avoiding tight clothing and losing weight if obese. People with athletes foot or tinea cruris can prevent spread by not lending their towels to others. Treatment
Tinea cruris is treated by applying antifungal medications of the allylamine or azole type to the groin region. Studies suggest that allylamines (naftifine and terbinafine) are a quicker but more expensive form of treatment compared to azoles (clotrimazole, econazole, ketoconazole, oxiconazole, miconazole, sulconazole). If the symptoms have been present for long or the condition worsens despite applying creams, terbinafine or itraconazole can be given by mouth.The benefits of the use of topical steroids in addition to an antifungal is unclear. There might be a greater cure rate but no guidelines currently recommend its addition. The effect of Whitfields ointment is also unclear, but when given, it is prescribed at half strength.Wearing cotton underwear and socks, in addition to keeping the groin dry and using antifungal powders are helpful. Prognosis and complications
Tinea cruris is not life-threatening and treatment is effective, particularly if the symptoms have not been present for long. However, recurrence may occur. The intense itch may lead to lichenification and secondary bacterial infection. Irritant and allergic contact dermatitis may be caused by applied medications. Epidemiology
Tinea cruris is common in hot-humid climates and is the second most common clinical presentation for dermatophytosis. It is uncommon in children. References
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