APPENDIX II: SYRINGOMYELIA

The principle features are:

• Headache- worsens with cough, sneeze, and strain.
• Neckache
• Pain in upper limbs, often exacerbated by valsalva manoeuvres, exertion or coughing.
• Areas of dissociated sensory loss, which may be in a bizarre distribution over the trunk and upper limbs.
• Loss of temperature sensation in upper limbs may lead to painless burns.
• Loss of upper limb reflexes; positive Babinski reflex
• Atrophy (wasting) of small muscles in the hands
• Spastic paresis, gradually progressive, leading to difficulty in walking. (increased muscle tone and weakness)
• Uncoordinated movements
• Muscle spasms and fasciculations (twitches)
• Skin rashes
• Alteration in sweating
• Raynaud’s phenomenon (cold, painful hands due to poor circulation)
• Horner’s syndrome (see above), nystagmus.
• Dysphagia (difficulty swallowing)
• Dysphonia (abnormal voice)
• Abnormal salivation.

(NB. These symptoms are sometimes seen in uncomplicated arachnoiditis. Jenik et al (xxv) stated that spinal cord syndromes due to non-traumatic adhesive arachnoiditis cause “predominantly syringomyelic sensory deficits.”)

Later stages may affect bladder, bowel and sexual function.

• Joint pains worse with straining.
• Charcot Joints (neurogenic arthropathy= joint damage due to lack of protective sensation)
• Symptoms may be unilateral or bilateral.
• An uncommon finding is onset of electric shock sensation running up and down the spine when the head is flexed or extended, occasionally followed by syncope (passing out). This is known as Lhermitte’s phenomenon.
• Some patients may show an increasing scoliosis (lateral curvature of the spine) which is thought to be due to unequal nerve supply to the paraspinal muscles.

Misdiagnoses have included:

• Carpal tunnel syndrome (neurological symptoms resulting from compression of the median nerve at the wrist)
• Ulnar nerve compression (ulnar nerve in the arm)
• Cervical spondylosis (degenerative disease of the cervical spine).
• Diagnosis is by MRI scan of the spine and EMG tests (electrical tests to detect muscle weakness)

Surgical treatment is usually necessary for symptomatic cases, and early intervention essential, if the syrinx is large and/or increasing in size, to avoid irreversible cord damage. Surgery may provide stabilisation or modest improvement in symptoms for most patients. Recurrence may necessitate further operations.

Shunting is used to drain the spinal fluid from the cavity into either the abdomen (syringoperitoneal) chest (syringopleural) or the subarachnoid space. This procedure carries risk of complications such as damage to the spinal cord, haemorrhage, infection, shunt blockage, low CSF pressure and spinal tethering.

A recent paper ([89]) suggests that all types of shunts may cause “significant morbidity” and lead to further surgical intervention.

A study specifically of syringomyelia secondary to arachnoiditis ([90]) found that outcome of surgery depended on the severity of the preoperative arachnoid pathology and that shunting was associated with recurrence rates of over 90%. For patients with focal scarring, microsurgical dissection of the scar and decompression of the subarachnoid space with a fascia lata graft stabilised over 80% of patients (but in cases with extensive scarring this was less than 20%).

Table of Contents

Introduction
THE SCALE OF THE PROBLEM
ARACHNOIDITIS OR EPIDURAL FIBROSIS?
NOMENCLATURE
THE INFLAMMATORY NATURE OF ADHESIVE ARACHNOIDITIS
PATHOLOGY
CLASSIFICATION
CAUSES
THE IATROGENIC ASPECT OF ADHESIVE ARACHNOIDITIS
PRESERVATIVES IN SPINAL INJECTIONS
PROGNOSIS
THE SYNDROMIC NATURE OF SYMPTOMS IN ADHESIVE ARACHNOIDITIS (Warning: LONG)
COMPLICATIONS OF ADHESIVE ARACHNOIDITIS
DIFFERENTIAL DIAGNOSIS
CLINICAL ASSESSMENT
DIAGNOSTIC TESTS
TREATMENT OPTIONS (Warning: LONG)
MULTIPLE CHEMICAL SENSITIVITY
LOOKING TO THE FUTURE
APPENDIX I: AUTOIMMUNE ASPECTS
APPENDIX II: SYRINGOMYELIA
NEXT: ADDENDUM - May 2000
REFERENCES