Successful endoscopic third ventriculostomy in noncommunicating thoracic syringomyelia.
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Haemangioblastomas involving the spinal cord are uncommon but by no means unknown. It is well established that haemangioblastomas of the central nervous system are commonly associated with lesions in the retinae and in various abdominal organs. The simultaneous occurrence of similar tumours in the lung is less common and not well recognized. The case of a man with a haemangioblastoma of the spinal cord with many small haemangioblastomas of the lung is reported here.
The present report covers an autopsied case of multiple hemangioblastomas in the cerebellum and the spinal cord, which did not involve the retinas and other organs. An intramedullary hemangioblastoma was found at the C3-4 level of the spinal cord and similar small tumors were present at the C-7 and Th-4 levels. Multiple cysts were located from the C1-2 to the lumbar level; some of these seemed to be longitudinally continuous, like syringomyelic cavities. These findings appear only rarely in published reports of autopsied cases of hemangioblastomas in the spinal cord.
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BACKGROUND: Although primary intramedullary tumors of the spinal cord with syrinx formation are well documented, there have been no reports of extensive syrinx formation or cystic degeneration associated with radiation necrosis. METHODS: We report a case of radiation necrosis and syrinx formation in a 49-year-old woman with a 5-year history of astrocytoma grade II of the cervical cord, who progressed to quadriparesis following surgery and radiation therapy. Magnetic resonance imaging (MRI) of the cervical and thoracic spine demonstrated enlargement of upper cervical cord (C1-C6) with diffuse increased signal enhancing mass by gadolinium, as well as appearance of syrinx from T4-T10. RESULTS: Autopsy findings indeed revealed a small, residual, infiltrating glioma in the upper cervical areas, but the diffuse parenchymal abnormality seen on MRI as prolonged T2 characteristics on double-echo spin-echo sequence was revealed to be radiation necrosis. CONCLUSION: What appeared to be a cystic cavity or syrinx at the thoracic level was also diagnosed as radiation necrosis with cyst formation on histologic examination.
A patient developed a painful progressive paraparesis following trauma. Metrizamide myelography revealed an arachnoid cyst. A delayed metrizamide computed tomography scan of the area confirmed the cyst and demonstrated a syrinx at that level that was not appreciated on the myelogram. The association of these two lesions after trauma are perhaps more common than appreciated and the importance of a computed tomography scan of the abnormal area on myelography is emphasized.
We recorded electrically stimulated somatosensory evoked potentials (electric SEPs) and pain-related SEPs following CO2 laser stimulation (CO2 laser SEPs) from a 17-year-old patient affected by myotonic dystrophy whose MRI disclosed a large syrinx extending from spinal level C2 to S3. Careful clinical and electromyographic examinations revealed no motor or sensory disturbances, apart from myotonia. The only abnormality noted in median and ulnar nerve short-latency electric SEPs (recorded with a non-cephalic reference electrode) was the absence of cervical component N13, the other SEP responses (N9, N10, N11, P14, N20) being normal. The cutaneous pain threshold and CO2 laser SEPs (both obtained by a CO2 laser beam applied to the back of the hand) were normal. Thus cervical component N13 appears to be highly sensitive to the effects of central cord lesions, even when these are asymptomatic.
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A 32-year-old male presented with progressive weakness and numbness of both upper limbs of one-month duration. The patient had weakness and wasting of small muscles of both hands with weak grip. Sensory system revealed graded sensory loss to pain, temperature and touch in C5 to T1 distribution and vibration and joint position sense from C5 to C8 in the both upper limbs. There was areflexia in the upper limbs while there was no motor or sensory deficit in the lower limbs. The cortical potential on stimulation of posterior tibial nerve was prolonged on both sides. On MR imaging of the cervical spine there was iso to low intense lesion which was hyperintense on T2-weighted imaging along the dorsal aspect of the cord extending from C2 to C6 level. The axial images showed involvement of the posterior column. The serum vitamin B12 level was found to be low. The patient responded to parenteral cyanocobalamine therapy and the radiological lesion subsequently resolved.
A 19-year-old immunocompetent man was admitted to hospital with diplopia, nausea, vomiting and change in mental status. The patient had a history of tuberculous meningitis that was diagnosed at another hospital 6 months before the present admission, and at that time anti-tuberculosis treatment was initiated using a first-line drug combination. A computed tomography (CT) scan of the brain revealed non-communicating hydrocephalus. A ventriculo-peritoneal shunt was inserted surgically. Two months later, the patient was hospitalized again for fever, dysphagia and left hemiparesis. At that time, his cranial CT findings were within normal limits; however, magnetic resonance imaging (MRI) revealed an irregular multilocular peripheral contrast-enhancing lesion in the posterior fossa. The abscess was surgically drained. The presence of acid-fast bacilli in the abscess material was demonstrated by Ziehl-Neelsen staining. Mycobacterium tuberculosis grew on Lowenstein-Jensen culture medium, and the strain was found to be resistant to isoniazid. One month after the operation, the patient became quadriparetic. Cervical MRI revealed a cervico-thoracic syringomyelitic cavity, after which a syringoperitoneal shunt was placed. Treatment with four drugs was continued for 10 months, and then treatment with three drugs for a total period of 18 months. The patient recovered, with residual quadriparesis. Even though very rare, isoniazid-resistant M. tuberculosis may be the causative agent of progressive tuberculosis.
Posttraumatic syrinxes may extend many cord segments rostral to a spinal cord injury (SCI) and significantly dilate the spinal cord, yet few neurologic deficits may be noted. Careful physical examination may reveal ascending loss of pain and temperature without evident functional motor decline. We present a 49-year-old man with T4 paraplegia and a large posttraumatic syrinx who died 3 weeks after syringoperitoneal shunting. Neuropathologic study revealed a large bilateral syrinx cavity from T1 to C6 that tapered to a small unilateral syrinx at C2. Light microscopy of sections from T1 to C2 showed massive loss of intermediate to intermedio-lateral gray neurons and moderate reduction of motoneurons at T1 to C6 levels. Despite these findings, manual muscle testing results remained normal for wrist extensors and elbow extensors, and the patient continued to perform independent sliding board transfers. We conclude that this large progressive syrinx did not merely dissect neural elements apart but caused extensive neuronal damage. Loss of interneurons was evident in spinal segments with preserved strength and function. Possible mechanisms to explain the relatively minimal clinical deficits in view of the neuronal loss are discussed.
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We report the clinical and MRI findings of two patients with familial spinal arachnoiditis. Although their initial symptoms were various, they both showed spastic paraparesis and sensory disturbance below the thoracic level. Cytokines and WBC in the CSF were studied, but they were not elevated at all. The spinal magnetic resonance images of each showed extensive arachnoiditis and a cystic structure. The other impressive features included: (i) an enhancement within the thickened arachnoid and an adhesion between the spinal cord and the dura mater, (ii) deformation of the thoracic cord where the arachnoid adhered, and (iii) secondary syrinx formation. Laminectomy may have an adverse outcome for such patients.