Treatment Oral copper supplementation, 2 mg/d for at least several months, seems effective in most patients, but some do not improve and the appropriate duration of treatment is unknown. Some patients relapse after an initial improvement even with continued administration or when copper supplementation is stopped. The gluconate, sulfate, or chloride preparations of copper may be used, although there has been concern about the bioavailability of the first-named compound. Intravenous therapy as a way of initially replenishing copper stores has been introduced but the need for such treatment is uncertain. Zinc supplements must, of course, be discontinued, as they lower copper levels.
There remains a group of subacute ataxic-spastic myelopathies that are not caused by multiple sclerosis or by B12 or copper deficiency. Progressive spastic or spastic-ataxic paraparesis of a chronic, irreversible type may also develop in conjunction with chronic, decompensated liver disease; with AIDS; in cases of adrenoleukodystrophy, particularly in the symptomatic female heterozygote; in tropical spastic paraplegia (HTLV-I); radiation myelopathy; and adhesive spinal arachnoiditis, which is discussed immediately below.
Spinal Arachnoiditis (Chronic Adhesive Arachnoiditis) (See Chap. 10)
This is now a relatively uncommon spinal cord disorder that was introduced in relation to the subject of low back pain in Chap. 10. It is characterized by a combination of painful root and spinal cord symptoms that may mimic intraspinal tumor. There is opacification and thickening of the arachnoidal membranes and adhesions between the arachnoid and dura—the result of proliferation of connective tissue. The subarachnoid space is unevenly obliterated. In this sense, the term arachnoiditis is not entirely appropriate, although it seems likely that the connective tissue overgrowth is a reaction to an antecedent arachnoidal inflammation. Some forms of arachnoiditis were traced to syphilis or to a subacute, therapeutically resistant meningitis of another type. Most others in the past followed the introduction of a variety of substances, most no longer used, into the subarachnoid space for diagnostic or therapeutic purposes or following spinal anesthesia, soon afterward or after an interval of weeks, months, or even years. This complication was eventually traced to a detergent that had contaminated vials of procaine.
More pernicious, however, is a delayed meningomyelopathy that developed within a few months or years of the inciting event, causing a spastic paralysis, sensory loss, and incontinence of sphincters. There are also cases on record in which an epidural or similar catheter has accidentally penetrated the cord and caused a traumatic partial myelopathy as mentioned earlier. Still seen regularly is a restricted form of arachnoiditis that complicates a series of operations for lumbar discs or the spinal injection of methylene blue. Less convincing are cases attributed to closed spinal injuries. In many cases, no provocative factor can be recognized. A familial form was reported by Duke and Hashimoto, but we have had no experience with it.
Clinical manifestations Symptoms may occur in close temporal relation to an acute arachnoidal inflammation or may be delayed for weeks, months, or even years as indicated above. The most common mode of onset is with pain in the distribution of one or more sensory nerve roots, first on one side, then on both, in the lumbofemoral regions. The pain has a burning, stinging, or aching quality and is persistent. Abnormalities of tendon reflexes are common, but weakness and atrophy, the results of damage to anterior roots, are less frequent. In thoracic lesions, symptoms of root involvement may antedate those of cord compression by months or years. Sooner or later, however, there is involvement of the spinal cord, manifest by a slowly progressive spastic ataxia with sphincter disturbances.