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PubMed2002

Thoracic paraganglioma presenting with spinal cord compression and metastases.

We report a case of a 41-year-old man with a primary thoracic spine paraganglioma presenting with spinal cord compression and distant metastasis. Paragangliomas arising within the spinal canal are rare and when they occur are usually found in the intradural, extramedullary compartment and in the lumbosacral region. Thoracic paragangliomas are very rare, with only five prior cases reported in the literature and with distant metastasis seen in only one of these. In this case, the tumor was entirely extradural and produced extensive sclerosis of the surrounding vertebrae. The clinical, radiographic, and pathologic features of this case are detailed and the relevant literature is reviewed.

Adult↗

PubMed1989

Tethered cord syndrome: a pediatric case study.

Tethered cord syndrome, seen in patients with spinal dysraphism, is a progressive neurological deterioration due to stretching of the spinal cord. Spinal dysraphism results from defects during embryonic closure of the neural tube, usually in the lumbosacral region. In tethered cord syndrome an abnormally low conus medullaris is tethered by intradural abnormalities such as a short, thickened filum terminale, fibrous bands, a lipoma or diastematomyelia. This article distinguishes tethered cord syndrome from other forms of occult spinal dysraphism and describes and compares normal anatomy to pathological changes. Clinical symptoms and nursing management of the pediatric patient with tethered cord syndrome will be exemplified by a case study presentation.

Child↗

PubMed2001

The retinoic acid-metabolizing enzyme, CYP26A1, is essential for normal hindbrain patterning, vertebral identity, and development of posterior structures.

The active derivative of vitamin A, retinoic acid (RA), is essential for normal embryonic development. The spatio-temporal distribution of embryonic RA results from regulated expression of RA-synthesizing retinaldehyde dehydrogenases and RA-metabolizing cytochrome P450s (CYP26). Excess RA administration or RA deficiency results in a complex spectrum of embryonic abnormalities. As a first step in understanding the developmental function of RA-metabolizing enzymes, we have disrupted the murine Cyp26A1 gene. We report that Cyp26A1-null mutants die during mid-late gestation and show a number of major morphogenetic defects. Spina bifida and truncation of the tail and lumbosacral region (including abnormalities of the kidneys, urogenital tract, and hindgut) are the most conspicuous defects, leading in extreme cases to a sirenomelia ("mermaid tail") phenotype. Cyp26A1 mutants also show posterior transformations of cervical vertebrae and abnormal patterning of the rostral hindbrain, which appears to be partially posteriorly transformed. These defects correlate with two major sites of Cyp26A1 expression in the rostral neural plate and embryonic tail bud. Because all of the Cyp26A1(-/-) abnormalities closely resemble RA teratogenic effects, we postulate that the key function of CYP26A1 is to maintain specific embryonic areas in a RA-depleted state, to protect them against the deleterious effect of ectopic RA signaling.

Abnormalities, Multiple↗

PubMed2006

Spinal tuberculosis: overlooked?

OBJECTIVE: The aim of the present study was to ascertain the degree of occurrence of tuberculous infection in patients presenting with low back pain (LBP). METHODS: Forty consecutive patients seeking primary medical attention for the main symptom of LBP and presenting to the Rheumatology Outpatient Clinic of our institution from January 2004 to June 2005, were recruited in this cohort study. All patients were thoroughly interrogated (occupational, trauma, infection, diabetes mellitus and medication history), subjected to a rigorous clinical examination and a battery of investigations RESULTS: Twelve of the 40 patients (33%) proved to have spinal TB as the cause of backache. Eight of these patients were above the age of 65. Five of the 12 patients with spinal TB also had concomitant osteoarthritic changes of the spine and two patients had concomitant disc prolapse. Eleven of the 12 patients with spinal TB had a completely normal chest X-ray and in 10 of these patients the plain X-ray of the lumbosacral region failed to show any significant lesion. In all 12 patients, sputum analysis failed to reveal acid-fast bacilli. CONCLUSIONS: The findings of this study indicate that TB is a common cause of LBP that is liable to be overlooked in the differential diagnosis of LBP. Furthermore, evidence of concurrent active intrathoracic TB may be lacking and consequently a high level of suspicion is required. The need for prompt diagnosis and treatment of skeletal TB is of utmost importance to prevent serious bone and joint destruction and severe neurological sequelae. A reliable imaging modality for diagnosing spinal TB seems to be magnetic resonance imaging.

Adult↗

PubMed2005

Cutaneous extraskeletal mesenchymal chondrosarcoma in a cat.

A 4-year-old, male cat was presented with a fixed, subcutaneous mass in the lumbosacral region. A histopathological examination revealed a well-defined but nonencapsulated neoplasm characterized by a proliferation of predominantly spindle cells, with high mitotic activity. Interspersed between these cells were single cellular elements with chondroid differentiation. Large areas of cartilaginous tissue with foci of endochondral ossification, necrosis and myxoid tissue were also observed within the neoplastic parenchyma. A diagnosis of extraskeletal mesenchymal chondrosarcoma was made based on the histological pattern - characterized by the coexistence of cartilaginous islands and undifferentiated mesenchymal cells, results of Alcian blue staining at various pH, immunohistochemical reactivity against vimentin and S-100, and the absence of skeletal involvement or other primary tumour sites. Clinical history of the cat excluded traumas, vaccinations or other types of subcutaneous inoculation. Six months on from surgical treatment, neither recurrence nor metastases have been detected.

Animals↗

PubMed1991

Diagnosis and treatment of neurogenic bladder due to partial sacral agenesis.

Fifteen patients with partial sacral agenesis were treated over a mean period of 68 months. Partial unilateral agenesis was present in 7 and bilateral sacral agenesis was present in 8. Three important diagnostic features are emphasised: symptoms referable to neurogenic bladder without an obvious neurogenic lesion (all 15 patients); skin or hair abnormalities in the lumbosacral region (9/15) and paraesthesia or other neurological disorders of the lower extremities (8/15). The definitive diagnosis can be made by careful inspection of a plain film of the lumbosacrum. Detrusor contractility was absent in 13 patients but preserved in 2; 11 patients were managed by clean intermittent catheterisation, 2 voided using detrusor contractility and 2 voided by means of the Valsalva/Credé manoeuvre. In 5 patients surgery in the intradural space preserved sexual function and prevented further deterioration in function of the lower extremities but could not restore bladder function that had already deteriorated. Complete continence was achieved in 11 patients and socially acceptable continence in 4. Early diagnosis and conservative treatment with clean intermittent catheterisation are extremely important in management. Should functional deterioration of the bladder and/or sensomotor function of the lower limbs occur, neurosurgery should be performed promptly.

Adolescent↗

PubMed1984

Alterations of amino acid transmitter systems in spinal cords of chronic paraplegic dogs.

The high-affinity uptake of [3H]serotonin, [3H]glutamate, and [3H]gamma-aminobutyric acid [( 3H]GABA) and the Na+-independent binding of [3H]glutamate and [3H]GABA were studied using spinal cord preparations obtained from normal mongrel dogs and from dogs made paraplegic by midthoracic spinal cord crush. Lumbosacral regions of the spinal cord were removed either before (1 week) or after (3 to 8 weeks) onset of spasticity. A myelin-free synaptosomal fraction was obtained by centrifugation and used for studying high-affinity uptake and for preparing synaptic plasma membranes for Na+-independent binding experiments. For the paraplegic groups, the uptake of 30 nM [3H]serotonin was 66 and 18% of control values after 1 and 3 weeks, respectively. Eadie-Hofstee analysis of [3H]serotonin uptake showed a 90% reduction in Vmax for the paraplegic group relative to control values, thereby indicating the expected loss of descending serotonergic pathways. The high-affinity uptakes of 1 microM [3H]glutamate and [3H]GABA were the same in both the control and nonspastic paraplegic groups after 1 week. However, after 3 weeks, the uptakes of [3H]glutamate and [3H]GABA were 60-70% higher for the spastic group than for the control animals. For both amino acids, Eadie-Hofstee plots revealed no difference in Km and higher Vmax for the spastic group relative to control values. After 1 and 3 weeks, the Na+-independent binding of 5 nM [3H]glutamate was 40-85% higher and the binding of 10 nM [3H]GABA was 40-60% lower for the paraplegic groups relative to the values for the control animals.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

PubMedDate not supplied

Skeletal carcinomatosis in endometrial clear cell carcinoma at initial presentation: a case report.

Bone metastasis is frequently seen in different solid tissue malignancies. However, it is a very rare entity in endometrial cancers and most of the previous reports were localized to a single bone. To the best of our knowledge, this is the first report of a case with disseminated bone metastasis from endometrial clear cell carcinoma. A 69-year-old, multiparous woman presented with the complaint of postmenopausal bleeding. Diagnostic workup revealed endometrial clear cell carcinoma. After comprehensive surgical staging, the patient was found to be at FIGO stage IIIC. Three weeks after the operation, an intractable back pain developed. Direct graphics of the lumbosacral region were suspicious of metastasis, and further evaluation of the patient revealed metastasis in multiple bones including calvarium, thoracolumbal vertebrae, pelvic bones, costae, collum of the right femur, and trochanter major of the left femur. Bone metastasis in endometrial clear cell carcinoma may be seen at initial presentation and may involve multiple bones. Whole-body scanning with scintigraphic evaluation is a reasonable approach to evaluate the extension of the bone involvement. Further studies are needed to elucidate the true incidence and management of bone metastasis in endometrial clear cell carcinoma.

Adenocarcinoma, Clear Cell↗

PubMedDate not supplied

Efficacy of an epidural combination of morphine and detomidine in alleviating experimentally induced hindlimb lameness in horses.

Amphotericin B-induced synovitis of the left tarsocrural joint was used to create a grade 3 of 4 lameness in 11 horses. Caudal epidural catheters were placed and advanced to the lumbosacral region. Baseline heart and respiratory rates were recorded and horses were videotaped at a walk and trot. Morphine sulphate (0.2 mg/kg) and detomidine hydrochloride (30 micrograms/kg) were administered to treated horses (n = 8) through the epidural catheter; an equivalent volume of physiologic saline solution was administered to control horses (n = 3) through the catheter. At hourly intervals after epidural injection for a total of 6 hours, heart and respiratory rates were recorded, and horses were videotaped walking and trotting. At the end of the observation period, video recordings were scrambled onto a master videotape. Lamenesses were scored by three investigators unaware of group assignment or treatment time. Lameness scores, heart rates, and respiratory rates were compared between groups using repeated measures analysis of variance. There was a significant decrease in lameness score after treatment with epidural morphine and detomidine (P = .0003); average lameness scores of treated horses were less than grade 1 at each hourly observation for 6 hours after drug administration. Early in the observation period, heart rates significantly increased in control horses and decreased in treated horses (P = .03). A similar trend occurred for respiratory rates (P = .07). Results of this study demonstrate that epidural administration of a combination of morphine and detomidine is capable of providing profound hindlimb analgesia in horses.

Adrenergic alpha-Agonists↗

PubMed1984

Sexual disturbances arising from multiple sclerosis.

Multiple sclerosis patients under the age of 50 with only a slight handicap were very often found to have disturbances of sexual functions (among male patients 71.4%; among female patients 73.9%; control group 18.7%). In 19.6% of the cases, the disturbances were of a serious nature. These disturbances, as well as disturbances of function of bladder and colon, were found to be mainly brought about by separate damage in the lumbosacral region of the spinal cord. The incidence of these disturbances bore no relationship to the degree of motor disturbances, age of the patient or duration of the disease.

Adult↗

PubMed1991

Murine infection by vesicular stomatitis virus: initial characterization of the H-2d system.

BALB/c mice and congenic H-2Ld-deficient BALB/c-H-2dm2 (dm2) mice were experimentally infected intranasally with isolates of vesicular stomatitis virus (VSV). The survival of infected hosts, viral replication in lungs and brains, and histopathologic in the two mouse strains were compared. In both strains of mice, mortality occurred during the period 7 to 10 days postinfection. However, dm2 mice were relatively resistant to lethal infections. Viral replication occurred at low levels in the lungs of both strains and did not evoke significant pathologic changes. In contrast, viral replication in the brains was much greater; in the BALB/c strain, this was accompanied by more frequent and more severe pathologic changes. In general, mice surviving at day 10 had effectively cleared virus from central nervous system but not respiratory sites. Evidence is presented that viral replication occurs first in the nasal cavity and is transmitted both to the lungs and to the olfactory bulb where focal cytopathology occurs. Virus enters the ventricles, causing encephalitis; necrosis occurs around the ventricles and in the lumbosacral region of the spinal cord. Necrotic lesions were accompanied by mononuclear infiltration. Mice immunized with virus of the same serotype or with a vaccinia virus hybrid encoding the VSV glycoprotein were protected from lethal infection; in contrast, mice immunized with heterotypic virus were susceptible to challenge.

Animals↗

PubMed1978

Analysis of evoked lumbosacral potentials in man.

Surface electrodes have been used to record potentials evoked in the lumbosacral region of 15 healthy volunteers after tibial nerve stimulation. By monitoring the M waves and H reflexes in the triceps surae muslces and by comparing the responses recorded over the roots with those over the lower cord, it was possible to identify the neural substrates responsible for several of the components in the responses. The findings are compared with those of previous studies in man and in other mammalian preparations.

Adolescent↗

PubMed1975

Electrocution of pigs.

Electrocution was considered to have caused 28 deaths in a house of 109 fat pigs. Post mortem examination showed haemorrhages at varying sites. Another 35, showing varying degrees of paralysis, were sent for slaughter. In most of these the pelvis was fractured and in some the vertebral column in the lumbosacral region. Twenty eight were condemned, 26 of them because of haemorrhages and because of spoilage associated with rupture of the urinary bladder.

Animals↗

PubMed1991

Intracellular study of electrophysiological features of primate spinothalamic tract neurons and their responses to afferent inputs.

1. Intracellular recordings were made from 43 spinothalamic (STT) neurons in the lumbosacral region of the spinal cord in anesthetized macaque monkeys. The antidromic responses of these neurons to electrical stimulation of the ventral posterior lateral (VPL) nucleus of the thalamus were examined, and orthodromic responses to electrical stimulation of the sural nerve or to mechanical stimulation of hindlimb skin were recorded to study the electrophysiological features of these neurons and their responses to afferent inputs. 2. The resting membrane potential of the neurons ranged from -26 to -70 mV and the antidromic latency from 2.3 to 9.1 ms. Three of the neurons were located in lamina 1 and were recorded so briefly that only antidromic and spontaneous activity could be studied. The rest of the neurons were located in laminae III-V and were of the wide-dynamic-range (WDR) type. 3. The antidromic action potentials recorded in the somas of STT neurons typically showed a fast rising phase and a short initial segment-somadendritic (IS-SD) delay. After repetitive antidromic stimulation, a progressive elongation of the IS-SD delay, widening of the spike, and failure of the SD spike were observed. 4. The afterpotential of the antidromic action potential consisted of a fast afterhyperpolarization (AHPf) and sometimes a delayed depolarization (DD) and a slow afterhyperpolarization (AHPs). The amplitude and the duration of the AHPs were progressively increased when longer trains of stimuli were used. When the membrane potential was hyperpolarized, the amplitude of the AHPs decreased, suggesting an involvement of K+ and/or Cl- ions. However, the AHPs completely disappeared when the strength of stimulation was adjusted to a level just below the threshold for the axon, suggesting that it was unlikely that recurrent inhibition contributed to the AHPs. 5. The background activity of 32 STT neurons was analyzed. The membrane potential at which spikes were triggered in these neurons was around -42 mV. The width and the rise time of the spontaneous spikes were shorter than those of antidromic action potentials, although the maximum rate of rise was similar. The heights of the spontaneous spikes were slightly shorter than those of antidromic action potentials. 6. Three types of background activity have been observed. One type had a very low average spontaneous rate with a bursting firing pattern, consisting of a few spikes superimposed on a depolarization. This type of activity was seen mostly in lamina I neurons. The second type of activity had a moderate to high spontaneous rate with a fairly constant interval between spikes.(ABSTRACT TRUNCATED AT 400 WORDS)

Action Potentials↗

PubMed2003

Tethered cord associated with anorectal malformation.

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.

Abnormalities, Multiple↗

PubMed2003

Spinal dysraphism: trends in northern India.

OBJECTIVE: To evaluate the clinical profiles, image findings and surgical outcome of 155 cases of spinal dysraphism. METHODS: 155 patients were studied prospectively (143) or retrospectively (12). The male to female ratio was 1.5:1. Mean age at presentation was 5.7 years. Out of 155 cases of spinal dysraphism, 119 had open spina bifida [meningomyelocele (MMC) in 113 (72%), meningocele in 3 (2%) and myelocystocele in 3 (2%)] and 36 had occult spina bifida [split cord malformation (SCM) without overt MMC sac (pure SCM) in 29 (19%) and midline dermal sinus in 7 (4.5%)]. Lipomeningomyelocele constituted 73 of the 113 cases of MMC (65%). Twenty cases of MMC (18%) had associated SCM (complex spina bifida). The total number of cases with SCM was 49 (32%). Twenty-four children with MMC presented with an operative scar from previous surgery, performed at the periphery. After a detailed clinical evaluation of all patients, craniospinal MRI was the preferable mode of investigation. All patients underwent surgery and were clinically assessed over a mean follow-up period of 3.6 years. RESULTS: MMC sac was the commonest skin manifestation seen in 89/155 cases (57%). Hypertrichosis and previous operative scar were noticed in 24 cases each (15%). Cutaneous hemangioma, skin tag and multiple neurofibroma were the other common superficial skin manifestations. Clinically, 103 patients (66.5%) had variable weakness of the lower limbs, and muscle atrophy was noticed in 56 cases (41%). Graded sensory loss, sphincteric dysfunction, trophic ulcer and backache were present in 89 (57%), 64 (36%), 17 (11%) and 9 cases (6%), respectively. Significant scoliosis in 56 cases (36%) and congenital talipes equinovarus in 51 cases (33%) were the most frequent neuroorthopedic deformities apart from high-arched foot, leg length discrepancy and flat foot. Common image findings were low-lying cord in 101 (65%), neural placode in 76/113 (49%), hydrosyrinx in 42 (27%), hydrocephalus in 71 (46%) and Chiari malformation in 62 cases (45%). The lumbosacral region in 56 cases (38%), followed by the lumbar region in 47 (30%), were the most common sites of occurrence of spina bifida. Of 71 hydrocephalic patients, 39 (55%) needed shunting before definitive surgery and 21 (30%) after the surgery. Eleven patients (15%) did not require shunting at all. CSF leak in 51 patients (33%), pseudomeningocele in 26 (17%), wound infection in 22 (14%) and meningitis in 13 (8%) were the most common postoperative complications. Two patients died in the postoperative period. During an average follow-up of 3.6 years (range 1.5-8 years), motor weakness improved in 47 children (45%) and remained static in 53 (52%), and 3 children showed deterioration in motor power. Sensory dysfunction improved in 43 (48%), remained static in 42 (47%) and deteriorated in 4 patients (4%). Sphincteric function clinically improved in 26 patients (41%) and was static in 38 (59%), and 6 patients (4%) had deterioration. Backache was dramatically relieved in all 9 patients with this complaint (100%). In 13 children with trophic ulcer (76%), it completely healed. CONCLUSION: A significant proportion of patients with open spinal dysraphism harbor an underlying SCM, an entity coined complex spina bifida (in our previous study). The incidence of hydrocephalus in spinal dysraphism is low in India in comparison to the Western world. Some patients with mild hydrocephalus on scan may not require a shunt operation. Surgical intervention should be early after entire neural axis screening by MRI.

Adolescent↗

PubMed2006

Pattern and factors affecting management outcome of spina bifida cystica in Ile-Ife, Nigeria.

BACKGROUND AND OBJECTIVE: There is paucity of data on the pattern and factors affecting the management outcome of patients with spina bifida cystica in the Ife-Ijesa zone, Nigeria. This study was designed to address this research question. METHOD: One hundred and six consecutive cases of spina bifida cystica who presented in our hospital from January 1990 to December 2004 were reviewed. We obtained information on sociodemographic factors, medical history and management as well as clinical outcome. SPSS was used to analyze the data. RESULT: Males constituted 54.7% and females 45.2% of cases. Mortality was high in those presented after the 4th week of life (p = 0.04). The malformation occurred in the lumbar and lumbosacral regions in 77.4%. Myelomeningocele was the most common type (86.8%). Hydrocephalus was recorded in 53.8% of patients. Surgical closure was done for 91.5% of the patients. About 77% of all the patients were discharged while 22.7% died. This was significantly related to age at presentation (p = 0.04) and infection before surgery (p = 0.045). Postoperative complications were more frequent in patients with ruptured lesions (p = 0.025), a larger size of defect (p = 0.028) and a lower birth weight (p = 0.006). CONCLUSION: Myelomeningocele is the most common type of spina bifida cystica in our environment. Late presentation and preoperative infection are associated with high mortality in our patients.

Abnormalities, Multiple↗

PubMed1992

Percutaneous trigeminal rhizotomy and percutaneous cordotomy under general anesthesia.

Most percutaneous procedures for the treatment of pain are done under local anesthesia and considerable pain can be involved. If the patient is elderly or confused because of severe pain, it is difficult or impossible to perform these procedures successfully. We treated 3 elderly patients with trigeminal neuralgia and 1 patient with severe bone pain due to metastatic osteosarcoma in the lumbosacral region by percutaneous trigeminal rhizotomy and percutaneous cordotomy under general anesthesia with intratracheal intubation. We conclude that percutaneous trigeminal rhizotomy and percutaneous cordotomy can be safely done under general anesthesia.

Aged↗