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[Diagnosis of spinal cord diseases using myeloscintigraphy].

Ninety-eight patients with a suspected spinal volumetric process were examined and treated. In addition to the routine examinations, modified myeloscintigraphy was performed in 48 patients in order to make an accurate diagnosis and to define process sites. Filling defect was the most common myeloscintigraphic symptom. This symptom was detected in 31 patients with spinal tumors and in 10 patients with another abnormality of the vertebral canal. Myeloscintigraphy was found to be of particular value in the diagnosis of spinal tumors. The technique allows minimum-size formations to be diagnosed even when there are no evidence for subarachnoidal space block.

Diagnosis, Differential↗

Chemosensitive epidural spinal cord disease in non-Hodgkins lymphoma.

Epidural spinal cord disease (ESCD), an infrequent complication of systemic non-Hodgkins lymphoma (NHL), can occur at diagnosis or at relapse, and is usually treated with radiotherapy, or infrequently surgical decompression. We retrospectively analyzed 140 patients with intermediate-grade NHL (IG-NHL) who were treated on a dose-intense protocol using doxorubicin, vincristine, and high-dose cyclophosphamide (NHL-15). There were seven episodes of ESCD in six (4.3%) patients. Five episodes were asymptomatic at presentation; one patient had back pain, leg numbness, and tingling; and one had radicular pain and mild leg weakness. None had malignant cells in the CSF. One patient received high-dose dexamethasone after laminectomy for diagnostic biopsy; otherwise, dexamethasone was used only as an anti-emetic prior to chemotherapy. Patients who developed ESCD at diagnosis received the planned course of NHL-15 chemotherapy as treatment for ESCD, and those treated with NHL-15 who developed ESCD at relapse were given a regimen containing ifosfamide, carboplatin, and etoposide (ICE). After chemotherapy alone, five of seven episodes showed radiographic resolution of ESCD and improvement of neurologic deficits. One patient received consolidation radiotherapy (2,700 cGy) to the spine after ICE for relapsed ESCD and had a complete response. One patient had progression of systemic lymphoma and ESCD despite chemotherapy. These data suggest that chemotherapy may be effective as initial treatment of ESCD in IG-NHL and may reduce the potential complications of spinal surgery and radiotherapy.

Adult↗

[Molecular pathogenesis of motor neuron diseases].

Spinal and bulbar muscular atrophy (SBMA) and amyotrophic lateral sclerosis (ALS) are representative motor neuron diseases in which selective neuronal degeneration occurs. In this paper, some molecular aspects are discussed related to the pathogenesis of the neuronal degeneration. SBMA is a an X-linked neurodegenerative disease caused by the expansion of a CAG repeat in the first exon of the androgen receptor (AR) gene. To date, eight CAG repeat diseases have been identified, including spinal and bulbar muscular atrophy (SBMA), Huntington's disease (HD), dentatorubralpallidoluysian atrophy (DRPLA), and five spinocerebellar ataxias (SCAs 1, 2, 3, 6, 7). These disorders very likely share a common pathogenesis caused by the gain of a toxic function associated with the expanded polyglutamine tract. Several mechanisms have been postulated as a pathogenic process for neurodegeneration caused by the expanded polyglutamine tract. In SBMA, nuclear inclusions (NIs) containing mutant AR protein have been observed in regions of SBMA central nervous system susceptible to degenerations. Transcriptional factors or their cofactors, such as CREB or creb-binding protein (CBP) sequestrated in NIs, may alter the major intracellular transcriptional signal transduction and ultimately may result in neuronal degeneration. The components in the ubiquitin-proteasome pathway also colocalized in NIs and contribute to the path-ogenesis of SBMA. We generated two types of transgenic mice expressing 239Q under the control of human AR promoter and full-size AR containing 97Q. Marked neurological symptoms and extensive nuclear inclusions were observed in both transgenic lines, but there was no neuronal cell death, suggesting that major neurological phenotype was due to neuronal dysfunction instead of neuronal cell death. As for the therapeutic strategies, the overexpression of Hsp70 and Hsp40 chaperones acted together to protect a cultured neuronal cell model of SBMA from inclusion formation and cell death by mutant AR with expanded polyglutamine tract. In regard to ALS, we are screening the gene expression profiles of the motor neurons from the human ALS and SOD transgenic mouse spinal cord. Motor neurons were microdissected from the spinal cord samples by a lazer-captured microdissection system. Gene expression profiles were screened by cDNA microarray and molecular indexing. Several new molecules were cloned and characterized for their function and relation to neuronal cell dysfunction. Some molecules characterized in this procedure were briefly described.

Amyotrophic Lateral Sclerosis↗

Scheuermann's disease or spinal osteochondrosis: its frequency and relationship with spondylosis.

Spinal osteochondrosis, previously known as Scheuermann's disease and spinal osteochondritis, is common and is probably becoming increasingly prevalent. Its incidence in patients whose primary complaint was of backache was shown to be twice as great as that in the general population. There was also a significantly higher incidence of lower lumbar spondylosis in patients with previous osteochondrosis compared with those without. Osteochondrosis is thereby shown to be an important aetiological factor in spondylosis.

Adolescent↗

Correlation between severity of clinical signs and motor evoked potentials after transcranial magnetic stimulation in large-breed dogs with cervical spinal cord disease.

OBJECTIVE: To evaluate use of transcranial magnetic motor evoked potentials for assessment of the functional integrity of the cervical spinal cord in large-breed dogs with cervical spinal cord disease. DESIGN: Randomized, controlled, masked study. ANIMALS: 10 healthy large-breed control dogs and 25 large-breed dogs with cervical spinal cord diseases. PROCEDURE: Affected dogs were allocated to 3 groups on the basis of neurologic status: signs of neck pain alone, ambulatory with ataxia in all limbs, or nonambulatory. Transcranial magnetic stimulation was performed on each dog with the same standard technique. Motor evoked potentials (MEP) were recorded from electrodes inserted in the tibialis cranialis muscle. Following the procedure, each dog was anesthetized and cervical radiography, CSF analysis, and cervical myelography were performed. The MEP latencies and amplitudes were correlated with neurologic status of the dogs after correction for neuronal path length. RESULTS: Mean MEP latencies and amplitudes were significantly different between control dogs and dogs in each of the 3 neurologic categories, but were not significantly different among dogs in the 3 neurologic categories. A linear association was evident between MEP latencies and amplitudes and severity of neurologic deficits; the more severe the neurologic deficits, the more prolonged the latencies and the more decreased the amplitudes. CONCLUSIONS AND CLINICAL RELEVANCE: Transcranial magnetic MEP are useful to assess severity of cervical spinal cord disease in large-breed dogs. Impairment of the functional integrity of the cervical spinal cord was found even in dogs with neck pain alone.

Animals↗

Outcome of late surgical treatment in patients with incomplete paraplegia due to spinal degenerative diseases.

STUDY DESIGN: Retrospective analysis. OBJECTIVE: To assess the outcome of late surgical intervention in patients with incomplete paraplegia due to spinal degenerative diseases. SETTING: Three men and four women with cervical or thoracic spinal degenerative diseases, who preoperatively were unable to walk for more than 6 months in Mie prefecture, Japan. METHODS: Review of clinical records and questionnaire survey regarding the walking ability of patients 2 years after surgery. RESULTS: All seven patients were unable to walk postoperatively. CONCLUSION: A late surgical intervention may not lead to functional recovery in patients with spinal degenerative disease who were unable to walk for at least 6 months.

Aged↗

Multiple thoracic paraspinal meningeal cysts in autosomal dominant polycystic kidney disease.

Spinal meningeal cysts have been reported in 3 patients as an extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). The authors report on a fourth patient with ADPKD who was found to harbor 7 thoracic meningeal cysts, appearing as paraspinal masses on plain films. The authors provide a comprehensive radiologic description of this abnormality.

Cysts↗

Spinal hydatid disease, a rare but existent pathological entity: case report and review of the literature.

Spinal hydatid disease is a not uncommon cause of spinal cord compression in endemic countries; however, involvement of the epidural space with sparing of the vertebral column is rare. Early diagnosis and surgical decompression with total removal of the hydatid lesion, when possible, is generally considered the standard of care for this disease. The authors describe a case of massive epidural hydatid disease without involvement of the vertebral column in a 62-year-old male patient, treated with a 2-stage surgical operation and administration of systemic albendazole. The literature is reviewed regarding the clinical features, diagnosis, treatment and prognosis of spinal echinococcosis.

Albendazole↗

Paraparesis (paraplegia), tetraparesis (tetraplegia), urinary/fecal incontinence. Spinal cord diseases.

Paraparesis (paraplegia) refers to partial (-paresis) or complete (-plegia) loss of voluntary motor function in the pelvic limbs. Similar involvement of all four limbs is termed tetraparesis (tetraplegia). Paraparesis generally results from spinal cord lesions caudad to the second thoracic spinal cord segment, whereas tetraparesis occurs because of lesions craniad to this segment (see discussion of spinal cord lesion localization in The Neurologic Examination and Lesion Localization, on page 328). The limbs may be affected equally; however, asymmetric lesions cause greater clinical involvement on the ipsilateral side. Strictly unilateral lesions at C1-T2 result in clinical involvement on only the affected side of the body (hemiparesis, hemiplegia). Monoparesis (monoplegia) occurs subsequent to unilateral T2-S1 lesions. Trauma and neoplasia are the most common spinal cord diseases affecting cats. Urinary and fecal incontinence often occur concomitant with paresis. General concepts relating to disorders of micturition are discussed at the conclusion of this chapter.

Animals↗

Home mechanical ventilation: investigation of 34 cases in Taiwan.

To evaluate the characteristics of and acceptance by persons in Taiwan who currently use positive-pressure mechanical ventilation at home (HMV), a survey was conducted in August 1992 through home care and equipment providers to investigate these patients. The 34 cases enrolled, including 18 males and 16 females, were classified into four groups: (1) lung disease group (LD) (n = 7) including chronic obstructive pulmonary disease and bronchiectasis; (2) neuromuscular group (NM) (n = 14) including diverse neuromuscular diseases; (3) spinal disease group (SP) (n = 7) including spinal cord injuries or tumors and (4) unresponsive stroke group (US) (n = 6). The average age of the SP group was much younger than those of the other three groups. The duration of home ventilation was from 1 to 36 months, with a mean duration of 10.6 +/- 8.7 months. Fifteen cases had received HMV for more than one year. Twenty six (76.5%) of the patients were completely dependent. All of the US patients had to be ventilated all day long in contrast to 71.4% of LD completely dependent. The cost of the US group was higher than that of the other three groups. Two of the LD, five of NM and one US patient had been re-admitted because of pneumonia, tracheostomy wound infection, granulation formation or weaning problems before the study. A follow-up survey was done four months, then one year later. The survival rate was 16.7% for LD group, 50% for NM and US group. Of the SP group, all patients were surviving at the end of this study. Most of the present HMV users were satisfied with their setting. It was concluded that HMV is an acceptable alternative way of therapy for long-term ventilation patients in Taiwan. SP and NM patients are better candidates for HMV. The overall one year survival rate is promising, while SP patients had the best prognosis. Patients who had already used HMV for more than 11 months, regardless of the disease, have a better chance to live another year.

Adolescent↗

[Spinal stiffness. Differential imaging diagnosis with reference to acquired hyperostosis syndrome and spinal loading disease].

There are numerous diseases which can irreversibly stiffen the whole spine or parts of the axial skeleton. Due to didactic reasons one can distinguish 3 groups of such disorders: Paradigmatic diseases of the spine with an inherent proneness to stiffness. Rare diseases which involve a certain danger of stiffening the spine. Rigidity of the spine due to scientifically unrecognised morphologic phenomena.

Diagnosis, Differential↗