JRSM >
Volume 99, Number 6 >
Pp. 321-322
Media |
1 Paediatric and Young Adult Orthopaedic Fellow, Royal National Orthopaedic
Hospital, Stanmore, Middlesex
2 Reader in Clinical Pharmacology and Honorary Consultant Physician, University
Department of Clinical Pharmacology, Radcliffe Infirmary, Oxford, UK
Correspondence to: Manoj Ramachandran#E-mail: manojorthopod{at}gmail.com
The Clubfoot by Jusepe de Ribera (1591-1652) in the Musée du Louvre in Paris (oil on canvas, 164x92 cm) has historically been thought to represent a hemiplegic disabled child begging for a living.1 It was painted in 1642 in Naples and was known as The Dwarf before it entered the collection of the Louvre in 1870. The work displays a young boy with apparent right-sided spastic hemiparesis, with a flexed wrist, an extended elbow, and an equinovarus deformity of the right foot and ankle. There is evidence of right-sided hemi-hypoplasia, with a shorter arm length and smaller girth of the right leg. With his left hand, he carries a crutch over his shoulder and holds a small piece of paper with the words Da mihi elimo / sinam propter / amorem dei (Give me alms, for the love of God), which some have suggested indicates mutism. However, there is no clear evidence for this.2 After all, modern beggars often display begging placards, and the artist has to show graphically what he cannot indicate by sound. Ribera used this technique in other paintings, for example his Blind Old Beggar, who holds a paper proclaiming Dies illa, dies illa. The boy has normal facial features with no evidence of dysmorphism or microcephaly. His smile and proud, upright posture are in keeping with Ribera's remarkable ability to capture the dignity of the individual, inviting respect rather than pity from the viewer.
Diagnoses that have been entertained in the past have included hemiplegic cerebral palsy, infarcts in the area of the middle cerebral artery, and the sequelae of congenital infection, such as rubella and cytomegalovirus.3 However, in the left upper limb, seen holding a crutch, ignored by previous commentators, there appears to be a flexion deformity of the wrist with an adducted thumb and extended elbow (although the latter could be as a result of shouldering the crutch). With involvement of three limbs and normal facies, the diagnosis of hemiplegic cerebral palsy seems unlikely. But these changes would be consistent with a diagnosis of arthrogryposis.
Arthrogryposis is from Greek words for a joint (arthron) and hooked (grupon), the latter referring to the hooked nails of the mythical griffin (grups). It is a condition defined by non-progressive contractures of two or more joints, often resulting from fetal akinesia.4 Its most common clinical features include symmetric contractures of all four limbs, tending to affect distal joints more than proximal, and fusiform atrophic limbs with absent skin creases. Intelligence is often normal and facial involvement is variable, the most common feature being micrognathia.
Arthrogryposis is, in fact, an umbrella term for a wide range of distinct
syndromes with specific phenotypes and characteristic gene defects. A more
accurate diagnosis here would be distal arthrogryposis, in particular type 1A,
where the clinical features include flexion contractures at birth with
predominant involvement of the hands (with adducted thumbs a prominent
feature) and feet (rigid equinovarus deformity), and variable expressivity
with asymmetric limb involvement. The face is not normally affected and
intelligence is normal. The features seen in Ribera's painting are consistent
with this diagnosis. The gene defect in distal arthrogryposis type 1A has
recently been characterized as an autosomal dominant mis-sense mutation
(Arg91Gly) on chromosome 9p21-q21 which affects tropomyosin 2, a protein
present in fast skeletal muscle
fibres.5
|
Arthrogryposis in its many forms has been documented in earlier societies in both prose and painting. A case of arthrogryposis multiplex congenita, most probably amyoplasia, which is the most common type of arthrogryposis, was described in an 8-year-old girl with hand and foot deformities by Thomas of Monmouth in a mid twelfth-century English hagiographic narrative, The Life and Miracles of St William of Norwich.6 A 16th century illustrated document, The Forme and Shape of a Monstrous Child, Born at Maydstone in Kent (1568), housed in the Huth Collection in the British Library, contains an anterior and posterior illustration of a newborn child with congenital deformities compatible with arthrogryposis, along with a poem and prose describing the anomalies.7 For example, the bilateral clubfoot anomaly is described as `the left leg growing upward toward the head, and the right leg bending toward the left leg, the foote thereof growing into the buttocke of the sayd left leg'. This particular case was seen in association with other congenital anomalies, such as genu recurvatum congenitum, cleft lip, and microcephaly, and is thought to be the earliest representation of Larsen's syndrome, one of the types of arthrogryposis.
It is therefore likely that arthrogryposis occurred in Spanish society in the 17th century, at the time of Ribera, and although the final diagnosis for The Clubfoot is speculative, arthrogryposis is certainly one worth considering.
Footnotes
REFERENCES
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
| ||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
