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Swollen and stiff shoulder

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Swollen and stiff shoulder

Q1: What are the pathological changes seen in the radiograph (see p 101)?

The radiograph shows destruction of proximal humerus and glenoid with joint effusion and no periosteal reaction. Neuropathic joint is one of the conditions in which such radiographic appearances are seen (box 1).

Box 1 : Differential diagnosis

  • Neuropathic joint (Charcot's joint).

  • Tuberculous arthritis.

  • Septic arthritis.

  • Primary and metastatic tumour.

  • Gorham's disease (vanishing bone disease).

Q2: What are the pathological changes seen in the mri scan (see p 101)?

The MRI scan shows Arnold-Chiari malformation with the herniation of cerebellum and medulla in the cervical canal region. A combination of central nervous pathology with bone destruction is consistent with neuropathic joint (Charcot's joint).

Discussion

Charcot's joint or neuropathic joint has been described as a disorganised and relatively painless joint. There are a number of causes of this condition (box 2) but no consensus regarding the pathogenesis. The neurotraumatic theory suggests that the changes result from repeated mechanical trauma to a joint that is insensitive to pain. The neurovascular theory proposes that the changes result from a neurally initiated vascular reflex that leads to hyperaemia and active bone resorption by osteoclasts.1 Forty years back, 90% of neuropathic arthropathies were related to tabes dorsalis, the remainder being distributed among the other disease entities. With the control of syphilis, diabetes mellitus and syringomyelia now account for a substantially larger proportion of Charcot's joints.2 Different aetiologies have predisposition to affect different joints in the body (box 3). Presently it is most commonly seen in the midfoot and forefoot of diabetic patients.

Box 2 : Causes of neuropathic joints

  1. Neural abnormality

    • Tabes dorsalis.

    • Syringomyelia.

    • Meningocele.

    • Spinal cord trauma.

    • Multiple sclerosis.

    • Paraplegia.

    • Arachnoiditis.

    • Adhesive arachnoiditis.

    • Arnold-Chiari malformation.

    • Charcot-Marie-Tooth disease.

    • Alcoholic neuropathy.

    • Leprosy.

    • Myelopathy of pernicious anaemia.

    • Congenital insensitivity to pain.

    • Familial amyloid neuropathy.

    • Non-familial amyloid neuropathy in dialysis patients.

    • Familial dysautonomia.

  2. Endocrine pathology

    • Diabetes mellitus.

    • Acromegaly.

  3. Infections

    • Leprosy.

    • Yaws.

  4. Drugs

    • Thalidomide.

    • Intra-articular steroids.

  5. Others

    • Calcium pyrophosphate dihydrate crystal deposition.

    • Amyloidosis associated with Waldenstorm's macroglobinuria.

    • Chronic haemodialysis.

    • Juvenile rheumatoid arthritis.

    • Scleroderma (with cervical osteolysis).

Box 3 Joints frequently involved in different conditions

  1. Diabetes mellitus

    • Tarsal and metatarsal joints.

    • Ankle joint.

  2. Tabes dorsalis

    • Knee.

    • Hip.

    • Spine.

  3. Syringomyelia

    • Shoulder.

    • Elbow.

    • Cervical spine.

  4. Calcium pyrophosphate dihydrate crystal deposition

    • Knee.

    • Hip.

Pain is most often the presenting feature, though it is not commensurate with the severity of the joint destruction. Some patients have no pain in the affected joints. Pain often increases with spontaneous fractures and dislocations. The joint is distended due to the effusion and synovitis. It is not inflammatory in nature, though in calcium pyrophosphate dihydrate crystal deposition disease it can be inflammatory. It may even be bloody or xanthochromic in nature at times.3

Radiographs show changes similar to those of osteoarthritis in the initial stages. But later on there is subluxation, fragmentation, and proliferation of bone with lot of debris in the joint. The bone destruction and joint disorganisation seen in radiographs should be distinguished from other causes of osteolysis (box 1). Bone scan shows increased uptake even in early disease. Pathological changes are similar to those of osteoarthritis but there is active pannus formation in Charcot's joints. Often the infection in these joints is difficult to diagnose. Delay in diagnosis of neuropathic joint is fairly common.4

Neuropathic shoulder is relatively a rare disorder and Arnold-Chiari malformation is an exceptionally rare cause of Charcot's joint. This is probably the fourth case of Charcot's shoulder due to Arnold-Chiari malformation.2

Treatment requires the management of the underlying condition if possible and the control of infection of the joint if present. Patient education has a major role in the management. They have to be taught regarding the use of protective footwear, braces, a cane, and immobilisation. Arthrodesis can be performed for unstable joints, though it is difficult and requires resection of great amount of bone. Total joint replacement is also not recommended because of lack of capsuloligamentous support, though they have been performed in hip and knee with success in 3–7 years follow up.5

Final diagnosis

Charcot's arthropathy of shoulder due to Arnold-Chiari malformation.

References

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