Split hand syndrome

Split hand syndrome is a rare neurological disorder characterized by a specific pattern of muscle weakness and wasting (atrophy) in the hands. This condition is also known as split hand/foot malformation (SHFM), ectrodactyly, or lobster claw hand.

Symptoms

The primary symptom of split hand syndrome is a specific pattern of muscle weakness and wasting in the hands. This typically affects the muscles that control the movement of the thumb and the index and middle fingers, causing these fingers to curl inward. The ring and little fingers are usually less affected. This pattern of muscle weakness and wasting can cause the hand to take on a claw-like appearance.

Causes

Split hand syndrome is caused by damage to the anterior horn cells in the spinal cord. These cells are responsible for sending signals to the muscles in the hands. When these cells are damaged, the muscles they control can become weak and waste away. The exact cause of this damage is not known, but it is thought to be related to a combination of genetic and environmental factors.

Diagnosis

Diagnosis of split hand syndrome is based on the characteristic pattern of muscle weakness and wasting in the hands. Additional tests, such as electromyography (EMG) and nerve conduction studies, may be used to confirm the diagnosis and rule out other conditions that can cause similar symptoms.

Treatment

There is currently no cure for split hand syndrome. Treatment is focused on managing symptoms and improving quality of life. This may include physical therapy to improve strength and flexibility in the hands, and occupational therapy to help with daily tasks. In some cases, surgery may be recommended to improve hand function.

See also

• Neurological disorders
• Muscular dystrophy
• Spinal muscular atrophy

References

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