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Three females with syringomyelia and atrophic shoulder arthropathy, in one patient associated also with hypertrophic elbow arthropathy and Morvan syndrome have been presented. Despite long duration and apparent symptoms the condition has been diagnosed only during hospitalization.
Somatosensory evoked potentials (SEPs) were elicited by magnetic stimulation of the tibial nerve, gastrocnemic muscle and by stimulation over the spinous processes of lumbar vertebra 5 (L 5), thoracic vertebra 9 (Th 9) and cervical vertebra 7 (C 7). The first SEP-positivity (P1-latency) was measured in a group of 20 controls and in another group of 18 patients with syringomyelia. The P1-latencies of the latter group following the stimulations over Th 9 and C 7 were significantly longer than those of the controls. Furthermore a correlation between electrophysiological findings and clinical data of the patients could be proven. Thus the method emerged as an appropriate tool for the investigation of the central part of the sensory system and for the diagnosis of spinal abnormalities.
INTRODUCTION: Based on medullary traction as responsible for idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID), Arnold Chiari malformation (ARCH), platybasia (PTB), basilar impression (IMB), odontoid recess (RTO) kinking of the brain stem (KTC) and considering the medullary traction to be transmitted by the filum terminale (FT), a surgical technique for the section of FT (SFT) is described in three cases of SMI, one of ESCID, and one of ARCH with no lumbar dysraphia. MATERIAL AND METHODS: A 34-year-old woman with cervico-brachialgias, paresthesias, bilateral babinski and a centro-medullary cavity C3-C7. A 26-year-old male with cervico-brachialgias, hypoestesia in left hemybody, and cervicobulbar cavity. A 19-year-old female with ESCID since the age of 14th, with episodes of reacuting, and 38o of dorsolumbar curvature. A 67-year-old woman with intense headache, hypoesthesia of the hands, paraparesia and ARCH. A 23-year-old man with marked tetraparesia, bilateral babinski, anesthesia of both legs, SMI, ESCID, ARCH and hydrocephaly. RESULTS: After SFT: in the SMIs the thermo-algesic, disesthetic and algic dissociation disappeared. In ESCID there was a reduction to 31o in the curvature in nine months. On ARCH the headaches ceased and there was recovery of touch and paraparesia. CONCLUSIONS: SFT is a useful etiological treatment for SMI, ESCID, ARCH. Also, in ESCID it is possible to avoid stress on the medulla due to its surgical reduction.
The paper is concerned with the results of myeloscanning of the subarachnoidal space in 32 patients with diseases of the spinal cord and its pia. The studies were conducted with albumin of human sera and labeled I131. The authors give a scannographical characteristics of the pathology of the subarachnoidal space in syringomyelia which included a change in the configuration, a deformation of the silhouette contour with marginal defects and focal irregular distribution of the isotope. In tumors of the spinal cord this method permits to defict the existence of a complete or partial block of liquor paths and the level of their occlusions. The author recommends a wider use of sabarachnoidal scanning when examining patients with lesions of the spinal cord and its pia pointing to the preciseness, simplicity and security of this method.
Extensive MR signal change in the craniad spinal cord parenchyma was found to be an ancillary sign of disease advancement in three patients with clinically progressive posttraumatic syringomyelia. This craniad margin of parenchymal spinal cord T2 hyperintensity resolved after cystoperitoneal shunt placement. There was a concomittant reduction or disappearance of the cyst in each instance, a halt in the progression of neurologic deficit, and some reversal of signs and symptoms.
Chiari type I malformation and so-called Chiari-I/syringomyelia complex continue to inspire controversy. Disagreement on these malformations concern both etiopathogenesis and treatment. Our still poor understanding of the natural course of the disease, the lack of consensus regarding therapeutic indications and even disagreement on appropriate surgery all contribute to cause disagreement. Significant progress has been made in our understanding of contributing etiopathogenetic factors in recent years. The most widely accepted hypothesis is that anomalous embryonic development characterized by paraxial mesodermal insufficiency would put volumetric constraints on postnatal development of the posterior fossa. We review historical and current controversies regarding Chiari type I malformation and classic theories on causative and contributing factors. We also discuss the latest surgical treatments that have been suggested, as well as associated anomalies--mainly syringomyelia, hydrocephalus and malformed cranial-cervical articulation. Finally, we propose a protocol useful for the diagnosis and treatment of Chiari type I malformation associated with dilation of the ventricular system.
We present the case of a young adult with type I Arnold-Chiari malformation (AMC1) and syringomyelia who developed central sleep apnoea and chronic respiratory failure, successfully treated with nocturnal noninvasive positive pressure ventilation ventilation (NIPPV). An extensive syringomyelic cavity (from bulbar to L4 segment) with severe impairment of the IX cranial nerve was documented and remains, although reduced, after the neurosurgical treatment. At baseline evaluation, the patient showed a moderate restrictive ventilatory defect, severe hypercapnic respiratory failure, abnormal control of breathing characterized by the absence of response to hypoxia and hypercapnia, and severe nocturnal central apnoeas. Nocturnal NIPPV was then started in the A/C mode with an improvement in blood gas values. Further evaluations were performed 10 and 18 months later. A progressive significant improvement of lung volumes, both in sitting and supine position, associated with a slight improvement of blood-gas values were observed. Nonetheless, the breathing pattern abnormalities persisted. Polysomnographic evaluation during mechanical ventilation showed a normalization of breathing pattern with arterial oxygen saturation (SaO2) > 90% throughout the night.
We report a series of 8 patients with syringomyelia and associated Chiari I malformation treated between 1992 and 1996 using a cranio-vertebral decompression with tonsil resection. The follow up period ranged from 6 months through 4 years (mean: 28 months). Neurological symptoms and signs improved in 7 patients (87%), and were stabilized in 1 (13%). At 6 months, postoperative MRI showed that the syringe has decreased in all patients. We emphasize the respect of the different stages of surgical procedure (limited foramen magnum opening, arachnoid preservation, tonsillar subpial aspiration, arachnoid suture and dural closure). Other of treatment and their results are reviewed.
An atypical case of post-traumatic syringomyelia is reported. Clinical presentation, electrophysiological and MR findings, as well as therapeutic efforts adopted for this case are discussed in the light of pertinent literature.
In 1970 a 20 year old woman presented with a pituitary chromophobe adenoma for which she underwent transfrontal pituitary surgery. In 1978 she had to be reoperated on because of local tumour recurrence, resulting in hypopituitarism. Bromocriptine (5 mg/day) was given for 15 years, but the plasma prolactin levels remained elevated. In 2000 the patient presented with signs and symptoms suggestive of a spinal cord lesion at the mid-thoracic level. A magnetic resonance imaging (MRI) scan showed an extensive leptomeningeal mass extending from the brainstem to L5, with a thoracic syringomyelia at the T7-T8 level. The plasma prolactin level was very high (5114 microg/l). A biopsy showed the presence of a metastasised prolactinoma. On administration of high dose cabergoline, 0.5 mg twice a day orally, the plasma prolactin levels decreased within one month and then normalised within 26 months. Tumour load reduced considerably but unfortunately, her signs and symptoms did not improve. This case illustrates that a high dose dopamine agonist might be an important therapeutic option in patients with a metastasised prolactinoma.
16 patients suffering from syringomyelia were checked for additional peripheral nerve lesions. On voluntary effort, often in conformity with the decrease of muscle strength, a moderate rarefication of the discharge patterns was apparent. Fasciculation potentials were common. Motor conduction velocities of the median and ulnar nerve were in the normal range in cases without additional peripheral nervous lesion at the elbow or the carpal. Depending on the severity of the peripheral nervous lesion, we found the duration of the muscle action potentials increased.
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