Syringomyelia is a degenerative spinal cord disorder characterized pathologically by the slow progression of cavity formation and glial hyperplasia within the damaged gray matter of the spinal cord. It most commonly affects the cervical and thoracic segments and may involve the brainstem and medulla oblongata. The primary clinical symptoms include dissociated sensory loss in the skin at the affected spinal segment level and long tract signs below the level of the lesion.

bubble_chart Diagnosis

1. Medical History and Symptoms:

Most commonly seen in young adults aged 20-30, with a male-to-female ratio of 3:2. Due to dissociated sensory loss on the body surface, patients often seek medical attention because of painless burns, cuts, or puncture wounds on their fingertips. As the condition progresses, muscle atrophy in the hands gradually appears, and upper motor neuron paralysis develops in the lower limbs.

2. Physical Examination Findings:

1. Sensory disturbances: Dissociated sensory loss in the area innervated by the affected spinal cord segment—loss of pain and temperature sensation with preserved touch sensation. Below the lesion level, tract-type sensory disturbances are observed.

2. Motor disturbances: Due to involvement of the anterior horn cells, atrophy and fasciculations occur in the small hand muscles (interossei, thenar muscles) and ulnar forearm muscles. Severe atrophy may result in a claw-like hand. As the disease progresses, atrophy may extend to other upper limb muscles, shoulder girdle muscles, and intercostal muscles. Below the lesion level, upper motor neuron paralysis manifests as increased muscle tone, hyperreflexia, and positive pathological signs.

3. Autonomic dysfunction: Damage to the lateral horn of the spinal cord leads to skin trophic disturbances, such as thickening, cyanosis, swelling, refractory ulcers, profuse sweating, or anhidrosis. If the lower cervical lateral horn is affected, Horner's syndrome may appear.

4. About 20% of patients develop joint damage. Due to loss of pain sensation, joints may undergo decalcification, abnormal mobility, and painless destruction, known as Charcot joints. If the lesion extends to the medulla oblongata, bulbar palsy may occur. Some patients may also present with scoliosis, pes cavus, basilar invagination, or hydrocephalus.

1. Lumbar puncture: Cerebrospinal fluid pressure and composition are usually normal in the early stages, but protein levels may increase in the late (third) stage.

2. Myelography with water-soluble contrast may reveal spinal cord widening.

3. Spinal CT or MRI can aid in definitive diagnosis. MRI, in particular, can exclude bone interference without requiring contrast injection, clearly displaying the location, morphology, and extent of the syrinx. It is currently the best method for diagnosing syringomyelia.

bubble_chart Treatment Measures

There is currently no specific treatment available.

I. Medical treatment:

1. Oral administration of 10% potassium iodide solution, 10ml three times daily.

2. Intrathecal injection of ¹³¹I IUCI (diluted with 5ml of cerebrospinal fluid and slowly injected into the subarachnoid space, once weekly). Some patients may experience symptom relief.

II. Surgical treatment:

For patients with concurrent atlanto-occipital malformation or arachnoid adhesions causing obstruction of the fourth ventricle outlet, as well as those with rapidly expanding syrinx leading to severe paralysis, decompressive drainage surgery may be attempted to alleviate symptoms. Localized syrinx may be considered for surgical resection.