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
