Dr David Bossingham is a retired rheumatologist and associate professor at James Cook University College of Medicine and Dentistry, Queensland.

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Simon was not able to offer much in the way of past medical history, but remembered missing a lot of school through illness, though he was unable to provide further details. Simon’ s loss of schooling meant he was barely literate or numerate.

He had been protected by his family all his life. His mother had recently died and this was likely a key factor in the timing of this latest presentation.

Assessment Simon had an unusual gait; he walked with small steps standing only on his toes and swayed from side to side. He was a large man, 185cm with a BMI of 32. There was something about his facies that struck his GP as unusual.

Close examination showed a globally restricted range of movement in his neck, with no pain.

Examination of his legs revealed a decreased range of movement in both hips, with marked muscle hypertonia.

All lower limb muscle groups were weak( grade 4) with exaggerated reflexes, a downgoing plantar response and clonus in both ankles. There was some weakness of hand and finger movements.

Further neurological examination revealed Simon

could not stand with his feet together and eyes closed, neither could he walk heel to toe. Hoffman’ s sign was positive( elicited by flicking the nail of the middle finger, causing the thumb of the same hand to flex and adduct).

Initial investigations, including blood tests, were unremarkable. Simon’ s GP referred him to a rheumatologist.

In addition to the findings above, the specialist identified that the unusual facial appearance was due to micrognathia. Simon also had very small hands and feet for such a large man. He had a spastic gait and muscle hypertonia causing restricted hip movement. Loss of hip flexion meant he could not sit normally on the clinic chair and looked most uncomfortable.

Investigations A plain X-ray of the cervical spine caused a flurry of excitement in radiology; there was fusion of the bodies of cervical vertebrae 3-6 both anteriorly and posteriorly and these vertebral bodies were smaller than their unaffected neighbours( see figures 1 and 2).

An MRI showed gross spinal stenosis in these segments that was worse still at C6-7 where the vertebrae were not fused.

Hip and pelvic X-rays showed remodelling of the femoral heads and loss of articular cartilage but no changes in the sacroiliac joints or lumbar spine. X-rays of the hands and feet showed the bones were disproportionately small, but that there were no joint changes.

Figure 1 and 2: Simon’ s cervical spine X-rays.

Diagnosis These findings are indicative of systemic onset juvenile polyarthritis or Still’ s disease. This condition causes severe systemic inflammation in a critical period of childhood that results in localised growth retardation in the vertebral bodies, hands, feet and jaw.

In Simon’ s case, in addition to restricting growth of the hands, feet and jaw, inflammation in the cervical spine had caused fusion of the affected bones, with associated spinal stenosis and cord compression. This was the cause of the upper motor neuron findings in the lower limbs, and positive Hoffman’ s sign, which occurs with cervical spinal cord compression.

Discussion George Frederic Still was a paediatrician who trained at King’ s College Hospital in London in the late 19th century. He described groups of children with arthritis of many forms, but this most pro-