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Peripheral primitive neuroectodermal tumor of the cauda equina in an elderly patient. Case report.

Primitive neuroectodermal tumors (PNETs) are aggressive neoplasms composed predominantly of undifferentiated cells that show evidence of neural differentiation. Although their classification remains disputed, PNETs are recognized as primary tumors in both the central and peripheral nervous systems. These rare tumors usually occur in children or young adults and are typically metastatic to the spinal cord. The authors report the case of a 70-year-old man with no significant medical history, in whom a peripheral PNET was demonstrated that, based on clinical and imaging findings, manifested as a nonspecific intrathecal tumoral process arising from the cauda equina. Although this lesion is rare in the elderly, the authors' case illustrates the importance of making this diagnosis as early as possible so that a treatment plan may be devised. The use of chemotherapy in association with radiotherapy in relation to the resection of the tumor should be reconsidered.

Aged↗

MRI of the cauda equina in CIDP: clinical correlations.

Isolated reports have documented enhancement and/or enlargement of spinal nerve roots on magnetic resonance imaging (MRI) in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This work examines those findings in a consecutive series of 16 patients with CIDP, with blinded comparison to MRI in 13 disease controls, including five patients with Charcot-Marie-Tooth disease type 1A. MRI sequences consisted of T1 weighted sagittal and axial views, before and after administration of gadolinium. Blinded MRI interpretation was performed independently by two neuroradiologists. MRI results were correlated with data collected from chart review. Enhancement of the cauda equina was seen in 11 of 16 CIDP patients (69%), and in none of 13 control subjects. Nerve roots were enlarged, most significantly in the extraforaminal region, in three CIDP patients, and in one patient with Charcot-Marie-Tooth type 1A. MRI findings did not correlate with disease activity and severity, nor with any clinical or laboratory features in patients with CIDP.

Adolescent↗

[Features of intracranial hypertension as a manifestation of cauda equina tumor].

A 61-year-old man was admitted to the Department of Neurology, Medical University of Białystok with signs of intracranial hypertension associated with headaches and papilledema, with no other signs of the nervous system dysfunction. CT and MRI brain scans were normal, cerebrospinal fluid contained 1620 mg/dl of proteins. One month later epileptic seizures with a loss of consciousness occurred. Brain CT and MRI scans showed no focal pathology and failed to disclose any intracranial tumor. The acute pain in the lumbar spine region made us perform an MRI scan of the lower spinal cord, which disclosed cauda equina tumor. The tumor was surgically removed and it was histopathologically concluded to be ependymoma. After the operation there were neither headaches nor seizures and we noticed an improvement in his vision acuity. In the postoperative course the patient suffered from urinary and bowel dysfunction. In this article we discuss a pathogenesis of intracranial hypertension occurring in spinal cord tumors and stress the need for a diagnosis of spinal cord lesions in patients with the elevated intracranial pressure.

Cauda Equina↗

Multiple neurilemmomas of the cauda equina, cavernous hemangioma of the spinal cord, and degeneration of the lateral corticospinal tracts in a man with the clinical diagnosis of multiple sclerosis.

Clinical and neuropathological findings of a 72-year-old man are reported who was thought to have had multiple sclerosis for 44 years. The main clinical symptom was a slowly progressive spastic tetraparesis, but during the last years he also developed bilateral neurogenic deafness, dysfunction of the urinary bladder and loss of vibratory and positional sensation in the legs. Autopsy revealed a unique combination of neuropathological findings, namely 1) multiple neurilemmomas of the cauda equina with loss of nerve fibers in dorsal roots and Goll's tracts, 2) symmetric fiber loss in the lateral corticospinal tracts throughout the spinal cord, and 3) a cavernous hemangioma of the lower thoracic spinal cord. No changes of multiple sclerosis were found. It is postulated that the patient may have had both neurofibromatosis and a sporadic or familial form of primary lateral sclerosis. The hemangioma in the spinal cord is probably unrelated to these two conditions, although it cannot be excluded that it represents a manifestation of von Recklinghausen's disease.

Aged↗

[Autonomic neuropathy with cauda equina syndrome in a patient with diabetes mellitus].

In significant number of diabetic patients are observed numerous complications during the disease onset and course. One of the most frequent is diabetic polyneuropathy that can, during its course, assume such types and imitate various diseases that can by the intensity of revealance be prominent and couse significant differential-diagnostic difficulties, especially if some forms of polyneuropathy are more rarely considered. The patient with poorly regulated diabetes, in whom the couse of disturbances was unknown for the longer period of time was presented in this study. Leading clinical phenomenology indicated the signs of impairment of cauda equina roots and extensive diagnostic procedures had to be performed. However, just after the favorable response to the glycemia regulation and withdrawal of neurologic phenomenology it was concluded that the patient suffered from diabetic polyneuropathy with clear signs of autonomic nervous system involvement, as well as autonomic neuropathy at the some time, respectively.

Autonomic Nervous System Diseases↗

The use of coaxially aligned freeze-thawed skeletal muscle autografts in the repair of the cauda equina--in the sheep.

The aim was to develop a model for study of nerve regeneration in nerve roots above the level of the dorsal root ganglion and to investigate the use of freeze-thawed muscle autografts for repair of nerve roots at this level. Four adult sheep were used for the experiment. A laminectomy was performed at the lumbosacral junction and the S2 root identified. Both the dorsal and ventral S2 roots were divided unilaterally within the dura and a freeze-thawed muscle graft was inserted into the nerve gap. When assessed at 6 months an action potential was recordable from the ventral root in one sheep. Histological examination of the nerve roots showed evidence of regeneration across the graft in the ventral roots of all the sheep and the dorsal roots of some. This preliminary work indicates a capacity for regeneration of the cauda equina and that freeze-thawed muscle can support this. It provides a useful model for further study of nerve root repair.

Animals↗

[A case of small epidermoid cyst in cauda equina with manifest regional hypesthesia].

This report concerns the 22-year-old female who has been suffering the paresthesia in the left buttock and thigh for three months. Neurological examination revealed nothing other than hypesthesia and hypalgesia which distributed in the third, fourth and fifth segments of left sacral region with the loss of anal reflex. Pain had not been noticed until when she came to our clinic. Myelography and MRI showed small irregular round mass occupying a third of the spinal canal behind the body of fourth lumbar spine. In the axial view of MRI, the mass was enhanced by the gadolinium (Gd) except for the small portion of its center. She underwent the surgical treatment which revealed the tumor entangling four nerves of cauda equina in its center as imaged in MRI. The tumor was epidermoid cyst which presumably caused the chemical meningitis and involved those nerves. That resulted in the sensory disturbance in the left sacral region, although the tumor was not large enough to compress the nerves.

Adult↗

Piriformis syndrome diagnosed by cauda equina action potentials: report of two cases.

STUDY DESIGN: Evaluation of recorded potentials from the lumbar spine after stimulation of the peroneal nerve in patients with piriformis syndrome is reported. OBJECTIVES: To report a new method of diagnosis of piriformis syndrome. SUMMARY OF BACKGROUND DATA: Clinically, the Freiberg and Pace signs were used to diagnose this entity. Among imaging examinations, computed tomography and magnetic resonance imaging have been used. An electrophysiological method using H reflex has also been reported. However, the best method for diagnosis of piriformis syndrome has yet to be clearly established. METHODS: After having an epidural electrode inserted into the lumbar spine, patients lay on their back. Cauda equina action potentials were recorded with the epidural electrode at L3/4 after stimulation of the peroneal nerve at the fibular head. As a control, the potentials were recorded with the hip and knee extended. Potentials were then also recorded with the hip flexed, and then with the hip flexed and internally rotated to stress the sciatic nerve by the piriformis muscle. RESULTS: We observed changes in amplitudes and latencies of recorded potentials. Amplitude decreased to about 70% of the control value in one case and to 67% in the other. On the nonaffected side, however, this amplitude did not decrease below 90%. The latency was also delayed on the affected side, but not on the nonaffected side. CONCLUSIONS: Recording of evoked potentials from the lumbar spine with stress to the affected leg was useful for diagnosing piriformis syndrome.

Action Potentials↗

Cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome): meta-analysis of outcomes after medical and surgical treatments.

The cauda equina syndrome in ankylosing spondylitis (the CES-AS syndrome) is marked by slow, insidious progression and a high incidence of dural ectasia in the lumbosacral spine. A high index of suspicion for this problem must be maintained when evaluating the patient with ankylosing spondylitis with a history of incontinence and neurologic deficit on examination. There has been disagreement in the literature as to whether surgical treatment is warranted for this condition. A meta-analysis was thus performed comparing outcomes with treatment regimens. Our results suggest that leaving these patients untreated or treating with steroids alone is inappropriate. Nonsteroidal antiinflammatory drugs may improve back pain but do not improve neurologic deficit. Surgical treatment of the dural ectasia, either by lumboperitoneal shunting or laminectomy, may improve neurologic dysfunction or halt the progression of neurologic deficit.

Adult↗

Cauda equina injury: late motor recovery.

A 21-year-old man was left with complete paraplegia and lower extremity sensory loss after a gunshot wound to the 4th lumbar vertebral body. Within three weeks of the injury he noted slight recovery of proximal left leg movements. With further motor recovery and rehabilitation, he was discharged ambulatory at nine months postinjury. The patient described late motor recovery developing 13 to 16 years after injury, particularly in right knee extensors and bilateral ankle plantar flexors, simultaneous with an aggressive home exercise program. Serial manual muscle tests revealed increased strength in hip flexors, knee flexors and extensors, and ankle plantar flexors. The patient progressed from ambulating with bilateral knee-ankle orthoses and forearm crutches to bilateral ankle-foot orthoses and a point cane. His original injury was a combination of neurapraxia, which recovered by resolution of conduction block in several weeks to months; axonotmesis, which recovered by axon regeneration over several years; and neurotmesis, which did not recover. The muscle strengthening achieved 14 years after cauda equina injury may represent strengthening of muscle fibers reiinervated after the initial nine-month rehabilitation program. Clinical implications for rehabilitation are discussed including the possible need for a therapeutic trial of muscle strengthening.

Adult↗

Ligation and partial excision of the cauda equina as part of a wide resection of vertebral osteosarcoma: a case report and description of surgical technique.

STUDY DESIGN: A case report. OBJECTIVES: To describe the unique challenges and novel surgical approach to treatment of vertebral osteosarcoma involving the dura as a margin. SUMMARY OF BACKGROUND DATA: Osteosarcoma of the vertebral column is a rare, malignant osseous tumor, carrying a poor prognosis. Currently, best available evidence supports that optimal surgical treatment entails wide excision of the tumor. Intentionally compromising neurologic function in order to ensure resection of the tumor with wide surgical margins can pose a difficult dilemma for the surgeon and patient. We describe here the first reported case, to our knowledge, of wide surgical resection of a vertebral osteosarcoma, including ligation and resection of part of the cauda equina and conus medullaris. METHODS: The clinical and radiographic presentations of a patient with osteosarcoma of L2 are presented. The challenges of surgical treatment of a primary malignant tumor of the spine, involving the dura as a margin, are discussed. The ultimate surgical technique employed to achieve wide surgical margins is described in detail. RESULTS: Four-year follow-up shows the patient is doing well, ambulating in a wheelchair, with no clinical or radiologic evidence of active disease or back or neuropathic pain and solid bony fusion. CONCLUSION: Currently, there is sufficient evidence to support the premise that the best chance for cure in sarcomas of the spine can be afforded through en bloc resection with negative margins. Neurologic forfeit in resection of spinal tumors, however, is usually at the root level, and this is the only case where such dramatic neurologic sacrifice was carried out. Although it is still early, the surgical and medical goals have been met, but most importantly, the patient's foremost goal of survival has been accomplished.

Adult↗

[Hemangioblastoma of the cauda equina--a case report (author's transl)].

We reported the case of a 40-year-old man who was hospitalized to our department on November 16, 1975 with a year history of neuralgia in the saddle region and vesicorectal dysfunction. Examination of CSF on lumbar puncture at L4-5 revealed xanthochromia with a protein content of 2,560 mg/dl. Myelography revealed a tortuous filling defect at the level of L2 coupled with complete blockade at L3 level. Selective spinal angiography of the left 12th intercostal artery demonstrated the enlarged Adamkiewicz's artery. This blood vessel was found to enter a diffuse uniform density, where no distinct blood vessels were visible. Within one second after the injection of contrast agent an efferent vein appeared from the right side of the density, ascended to the level of Th12 and then, upon turning, descended to the pelvic canal. These findings suggested a hemangioblastoma of the spinal cord. Laminectomy was performed at levels of L2 through L4 and a hemangioblastoma of the cauda equina was totally excised at L3 level. Although the postoperative course was complicated by a transient dysuria and hypesthesia at S1-S5 levels, complete cure was achieved in a month. In the present paper clinical features and neuroradiological examination of a hemangioblastoma of the spinal cord were described.

Adult↗

[Neurinoma of the cauda equina associated with normal pressure hydrocephalus].

Hydrocephalus associated with spinal tumors is a well known but not common phenomenon. Over 80 such cases have been reported since 1931 by Kirieleis. Most of those cases present features of raised intracranial pressure, for instance papilledema. The histological diagnosis of the tumors is ependymoma (1/2), neurinoma (1/4) and malignant astrocytoma (1/4). On the other hand dementia as a symptom of spinal tumors has been reported only 13 times. In 11 of 13 cases presented with signs of NPH, mental status improved only by removal of the tumors without shunt operation. The histological diagnosis of the tumors is neurinoma (9 cases), ependymoma (2) and meningioma (2). Ours is the 14th case. A 59-year-old woman presented a 6 month history of memory disturbance, gait disturbance and urinary incontinence. CT scan showed ventricular dilatation. RI cisternography revealed a block at lumbar region. CSF obtained by lumbar puncture was deep yellow and turbid. CSF protein level was markedly elevated to 4073 mg/dl. MRI of the spinal cord demonstrated cauda equina mass. Total removal of the spinal tumor was performed. The histological diagnosis was neurinoma. Within 3 weeks the patient's symptoms had dramatically improved. The decrease in ventricular size was noted on CT 18 months after operation. We think one of the most important factors by which spinal tumors develop NPH is elevation of the CSF protein content. In our case, postoperative protein level decreased from 4073 to 274 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)

Cauda Equina↗

Normal pressure hydrocephalus associated with cauda equina neurinoma.

A 65-year-old woman with normal pressure hydrocephalus experienced improved gait and hydrocephalus following surgical resection of a cauda equina neurinoma. Fibrinogen, detected in the cerebrospinal fluid, may be involved in the pathophysiological process of normal pressure hydrocephalus.

Aged↗

[Chronic fibroplastic leptomeningitis of the spinal cord and cauda equina].

Chronic (seldom subacute) low-grade spinal leptomeningitis, cystic and adhesive in natura, is a clinicopathologic condition known by several names in medical literature (arachnoiditis, arachnoditis adhesiva circumscripta, a.s.o.). The cystic structures are not cysts in the true sense of the word. They are in reality collections of cerebrospinal fluid walled off by meningeal adhesions. The extent of the leptomeningitis varies from a well-localized lesion to a diffused process involving the entire cereprospinal axis. In some cases the chronic proliferative changes lead to calcification and ossification of the leptomeninx. As well the circumscribed adhesive as the cystic form can produce a spinal cord compression syndrome similar to a tumor or a hydro-syringomyelia. Progressive paraparesis with difficulties of bladder and bowel function may nevertheless be arrested, or spontaneous remission may occur, perhaps due to temporary improvement of the local circulation of spinal fluid. An almost typical sciatic syndrome can be occasionally associated with a thickening of the leptomeninx and cystic spinal fluid collection in the region of the cauda equina. The lumbar adhesive and cystic form is as a rule a more benign disease then leptomeningitis occurring at thoracic or cervical levels. The etiological factors are trauma, spinal anesthesia, myelography, blood in the subarachnoid space, previous operations and infections. When the origin of the illness remains unidentified, low grade bacterial or viral infections can be presumed. We operated 2 patients with circumscribed severe leptomeningitis several segments far away from an intradural meningeoma. Such illness is uncommon, but not very rare. From 1970 until the end of 1986 we have carried out 33 laminectomies on 26 patients because of spinal leptomeningitis among 603 patients operated because of spinal cord compression (4.31%). Our cases are presented and literature is discussed. The dilemma what we have to do with a patient with symptomatic spinal leptomeningitis is open: the microsurgery can help the patient significantly, even if complete recovery is rare. In view of the extreme variety of these lesions a circumspect, sometime skeptical analysis of each patient must precede decision about surgery. We try to give some indications for microsurgery.

Adolescent↗