Syringomyelia and Arnold-Chiari syndrome

Many hypotheses concerning pathogenesis of syringomyelia were abandoned because of evidence found in more recent investigations. We should rank among them the "classical" theories of Gardner and Williams based on the assumption that syringomyelic cavities result from directing the fluid from the fourth ventricle to the central canal of the spine in the case of disturbances of circulation of the cerebrospinal fluid in the region of the cranio-spinal junction. The theory of intraspinal pulsation pressure of Greitz may explain the pathogenesis of syringomyelia in the case of obstacles to fluid flow from the cranial cavity to the spinal canal as in patients with Arnold-Chiari syndrome. The origin of Arnold-Chiari syndrome is connected with narrowness in the posterior fossa, particularly with narrowing of the arachnoid spaces. Improvement of clinical condition after surgical restoration of the fluid spaces within the posterior cranial cavity and improvement of cerebrospinal fluid flow in the region of the cranio-cervical junction are factors supporting this opinion.     

Tuberculous optochiasmatic arachnoiditis

Arachnoiditis involving optic nerve and the optic chiasm can occur as a complication of tuberculous meningitis (TBM). This study evaluates the clinical features, cerebrospinal fluid (CSF) and laboratory parameters and imaging findings of optochiasmatic arachnoiditis (OCA) and also tried to identify any factors which can predict this complication in patients with TBM. Patients admitted with TBM in the neurology wards of a tertiary care teaching hospital over a period of 6 years formed the material for this study. Student's "t" test and univariate analysis were done to identify any predictors for this complication and the variables found to be significant were further analyzed by multivariate logistic regression analysis. One hundred sixty-three patients with TBM, admitted over a 6-year period, were studied. Twenty-three (14%) patients developed OCA. Eighteen out of 23 (78%) developed this complication while on antituberculous treatment (ATT) and 5/23 (22%) were newly diagnosed cases of TBM. Of those already on treatment, 12/23 (52%) were receiving only ATT, the remaining 6/23 (26%) had received steroids along with ATT in varying doses and duration. The average period from diagnosis of TBM to visual symptoms was 6.4 months. On the multivariate logistic regression analysis, female sex (P < 0.037), age less than 27 (P < 0.008) years and protein content in the CSF > 260 mg% (P < 0.021) were the factors predisposing toward this complication. At 6 months follow-up, on treatment with steroids and ATT, 17% had improvement and no further deterioration was noted in visual acuity in 52%. OCA can develop even while on treatment with ATT. Young women with a high CSF protein content seem to be more prone for this complication.     

Recurrent spinal adhesive arachnoiditis. A case report

Spinal adhesive arachnoiditis is not an uncommon disease, usually having a monophasic course. We studied an atypical patient with recurrent spinal adhesive arachnoiditis nine years after intrathecal anesthesia and the first attack of the disease. Also noteworthy was the favorable evolution after surgery.     

Syringomyelia and its surgical treatment--an analysis of 75 patients.

A consecutive series of 75 patients with syringomyelia is presented, all of whom were treated by cranio-vertebral operations. Attention is drawn to the difficulty in assessing the results of treatment but 56 stabilised or showed modest improvement after surgery. Occluding the central canal appeared to have no greater influence on the progression of the disease than did simple decompression and did have a higher incidence of complications. Upper motor neurone weakness, joint position sense and central neck pain are the features most likely to improve and it is concluded that relieving the medullary compression resulting from a Chiari type 1 malformation, rather than influencing the syrinx, is the means by which this may occur. Simple decompression with preservation of the arachnoid membrane, combined with syringostomy in certain cases, is recommended.     

Syringomyelia as a complication of tuberculous meningitis.

Tuberculous meningitis may rarely be followed by the development of syringomyelia despite appropriate chemotherapy. In the present paper, we report a case of tuberculous meningitis in a 23-year-old Vietnamese male complicated by a rapidly progressive myelopathy due to granulomatous arachnoiditis which culminated in the development of a syrinx. The relevant literature is reviewed. The present case supports the hypothesis that vasculitic thrombosis of spinal cord vessels leading to ischemic myelomalacia is the mechanism causing postinflammatory syringomyelia.     

Noonan's Syndrome with Syringomyelia

Abstract: A case of Noonan's syndrome with cervical syringomyelia is presented here representing the second reported instance of such association. A CT of metrizamide myelography revealed a high density in the middle subintradural space in the cervical region below C₂. Sensory dissociation was minimal and localized in the left C₄. It could not be determined whether this association between syringomyelia and Noonan's syndrome is related or represents a chance.     

Syringomyelia: Electrophysiologic aspects

The clinical and EMG findings in 44 patients with syringomyelia who were seen at the Mayo Clinic between 1976 and 1985 are presented. In 10 of the patients, somatosensory evoked potentials (SEPs) of the upper and lower extremities were obtained. All 44 patients had radiographic or surgical evidence of a cervical syrinx. The most common abnormality on nerve conduction studies was a reduced hypothenar compound muscle action potential amplitude (23 patients). Abnormal findings on needle electromyography were present in 33 patients and included sparse fibrillation potentials, reduced motor unit potential (MUP) recruitment, and chronic neurogenic MUP changes in muscles innervated by the C-5, T-1 roots, with the most pronounced changes in small hand muscles. Ulnar and median nerve SEPs were usually normal in the presence of a dissociated sensory loss and were usually abnormal when all sensory modalities were impaired. Abnormalities of tibial nerve SEPs were frequent and were related to impaired proprioceptive sensation in the lower extremities.     

Syringomyelia presenting as ulnar neuropathy at the elbow.

OBJECTIVES: Syringomyelia may present with confusing, unilateral patterns of segmental muscle involvement and dissociated sensory loss. The objective of this study was to report a patient with Chiari malformation type 1 (CM1) and syringomyelia who had an unusual presentation suggesting ulnar neuropathy at the elbow. RESULTS: A 24-year-old woman presented with clinical evidence of ulnar neuropathy at the elbow except that there was disproportionate abductor digiti mini (ADM) atrophy and weakness, equivocal ipsilateral abductor pollicis brevis weakness and hyporeflexia in both arms. Nerve conduction studies revealed marked amplitude reduction of the left ulnar ADM-compound muscle action potential (ADM-CMAP) with a normal first dorsal interosseous-CMAP amplitude, no focal slowing or conduction block, and a normal ulnar sensory response amplitude. Electromyography (EMG) showed multi-segmental, left C7-T1 fibrillations and chronic reinnervation changes. Magnetic resonance imaging (MRI) of the cervical spine demonstrated CM1 and syringomyelia. CONCLUSIONS: Syringomyelia may clinically mimic ulnar neuropathy at the elbow.