Handigodu Syndrome

Handigodu Syndrome (HS) or Handigodu Joint Disease (HJD) is a crippling disorder endemic to Malenadu (Malnad) region in southern India. It is named after the village of Handigodu, where it was first noticed, in Shimoga district of the state of Karnataka*. Though not clinically identical, HS is akin to Mseleni Joint Disease in the Zululand of South Africa.

Prevalence of HS in the general population may not be meaningful as it is restricted to a specific geographic region. However, it is known to have killed over 1000 people since it was first noticed around 1975.

HS is an inherited degenerative osteoarthropathy (bone and joint disease). In this progressive skeletal system disorder, affected individuals are presented with severe joint and hip pain. In certain extreme cases in adults, crippled subject are made to crawl due to pain. Lack of normal growth is noticeable from early childhood, such as deformation of the limbs and dwarfism. Symptom onset is at preadolescence (as early as age 10) to young adulthood. Difficulty in walking and pain in hip joints eventually affect HS patients' locomotion and nominal routine chores. On the basis of anthropometric and X-ray analysis, HS is subdivided into three groups:

Type 1: Osteoarthritic changes in hip joints and average patient height.

Type 2: Dysplastic changes in hip and spine and short statured. Arm span exceeds patient's height.

Type 3: Spine involvement is severe and dwarfism.

Genetically HS is defined as autosomal dominant inheritance, requiring only one of the two inherited genes to be defective for disease onset. The cause of HS is yet to be defined. The proposition that environmental factors and consanguinity (marriage among close relatives) may play some role in HS appear to be speculative in nature. HS patients are treated for their pain but have no cure.

*HS was initially noticed in 1975 by a social worker, H. M. Chandrashekhar, in Handigodu according to one report. National health research organizations such as Indian Council of Medical Research (ICMR), National Institute of Nutrition (NIN), National Institute of Mental Health and Neurosciences (NIMHANS) and direct Government involvement are aiming to root out the malady. HS cases have been reported from Sagar taluk of the Shimoga District, and Sringeri, Koppa, and N.R.Pura taluks of Chikmagalur District. The Chanangi and Chaluvadi are the two lower socioeconomic communities in which the HS is predominantly seen. HS is not observed in higher or other lower socioeconomic groups in the same area. Research and treatment centers for HS are located in Shimoga and Balehonnur. Research on HS is very limited and all available scientific literatures are listed below.

References and further readings

Bhat RV, Krishnamachari KVAR. Endemic familial arthritis of Malnad. Indian J. Med. Res. 1977;66:777–86.

Krishnamachari KVAR, Bhat RV. Endemic familial arthritis of Malnad. An outbreak in Southern India. Trop. Geogr. Med. 1977;30:33–37.

Agarwal SS, Phadke SR, Phadke RV, Das SK, Singh GK, Sharma JP, Teotia SP, Saxena BN. Handigodu disease: a radiological study. A new variety of spondyloepi(meta)physeal dysplasia of the autosomal dominant type. Skeletal Radiol. 1994;23:611-9.

Agarwal SS, Phadke SR, Fredlund V, Viljoen D, Beighton P. Mseleni and Handigodu familial osteoarthropathies: syndromic identity? Am. J. Med. Genet. 1997;72:435-9.

Dhariwal AC, Bhattacharjee J, Sharma RS. Handigodu disease: A disease of unknown etiology - revisited. J. Commun. Dis. 1998;30:193-6.

Badadani M, Shetty KT, Babu SV, Agarwal SS. Metabolic status of magnesium and ceruloplasmin in Handigodu Joint Disease: a variety of spondylo epi (meta) physeal dysplasia.Clin. Chim. Acta. 2008;395:170-1.

Badadani M, Babu SV, Shetty KT, Agarwal SS. Peptide bound hypohydroxyprolinuria in Handigodu Disease: a familial syndrome of spondylo epi(meta)physeal dysplasia. Dis. Markers. 2009;27:7-12.

Contributor: Duraiswamy Navaneetham PhD.

Temple University School of Medicine

Philadelphia, PA, USA

November 2009