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A method of calculating spinal cord transit time from potentials evoked by tibial nerve stimulation in normal subjects and in patients with spinal cord disease.

Somatosensory potentials were evoked by stimulation of the tibial nerve at the ankle and recorded over the spine and scalp in 16 normal subjects and 26 patients with known or suspected spinal cord disease, with the aim of developing a method of measuring spinal sensory conduction velocity using a tolerable number of stimuli, applied unilaterally to alert subjects. In normal subjects N21 was consistently recorded over L1 vertebra and in most subjects a complex, N27/N29/P33, was recorded over the cervical spine referred to the vertex. Constant latencies at different spinal levels and, in one subject, comparison with the latency of the ascending volley indicate that the complex was not derived from the spinal cord but from more rostral structures, and therefore only transit time, rather than velocity, could be measured. In patients with clinically definite multiple sclerosis, even with minimal clinical signs, the N27/N29/P33 complex was always abnormal. Abnormalities in this and other forms of spinal cord disease were commonly absence or distortion of the complex, prolonged transit time being rare. The clinical value of the method is limited by the very low amplitude of the responses.

Adolescent↗

Spinal hydatid disease: a case series.

BACKGROUND: Over the past 10 years, 4 cases of spinal hydatid disease (3 men, 1 woman) were diagnosed and treated at our institution, with an average follow-up of 4 years. Hydatid disease of the spine is a rare condition with a poor prognosis that presents diagnostic and therapeutic challenges. METHODS: The patients were evaluated clinically, using the latest imaging modalities available in our institution. Decompressive surgeries were performed and the diagnosis was confirmed by histopathologic examination. All patients received long-term antihelminthic therapy with 400 mg of albendazole 3 times daily for 1 year. RESULTS: After surgery, all patients improved; however, over time, recurrence and residual disease were observed. Two patients had complete neurologic recovery at follow-up at 2 to 3 years, although there were radiographic signs of recurrence. The other 2 patients did not achieve complete neurologic recovery despite anterior decompression; they developed recurrent disease and the neurologic status deteriorated to spastic paraplegia. All patients refused further surgeries for recurrences and 2 patients died of complications of paraplegia. CONCLUSION: Diagnosis was challenging, eradication was difficult, and hydatid disease recurred in all 4 patients. In our experience, morbidity and mortality were high and prognosis was poor.

Adult↗

Pharmacological induction of heat-shock proteins alleviates polyglutamine-mediated motor neuron disease.

Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease caused by the expansion of a trinucleotide CAG repeat encoding the polyglutamine tract in the first exon of the androgen receptor gene (AR). The pathogenic, polyglutamine-expanded AR protein accumulates in the cell nucleus in a ligand-dependent manner and inhibits transcription by interfering with transcriptional factors and coactivators. Heat-shock proteins (HSPs) are stress-induced chaperones that facilitate the refolding and, thus, the degradation of abnormal proteins. Geranylgeranylacetone (GGA), a nontoxic antiulcer drug, has been shown to potently induce HSP expression in various tissues, including the central nervous system. In a cell model of SBMA, GGA increased the levels of Hsp70, Hsp90, and Hsp105 and inhibited cell death and the accumulation of pathogenic AR. Oral administration of GGA also up-regulated the expression of HSPs in the central nervous system of SBMA-transgenic mice and suppressed nuclear accumulation of the pathogenic AR protein, resulting in amelioration of polyglutamine-dependent neuromuscular phenotypes. These observations suggest that, although a high dose appears to be needed for clinical effects, oral GGA administration is a safe and promising therapeutic candidate for polyglutamine-mediated neurodegenerative diseases, including SBMA.

Animals↗

[The fulminant meningeosis blastomatosa of a medulloblastoma during postoperative chemotherapy].

BACKGROUND: Chemotherapy is currently investigated in young children with medulloblastoma with the objective to omit or even delay craniospinal irradiation. CASE REPORT: We report a case of very early meningeal progression of medulloblastoma during postoperative chemotherapy in a 2.5-year-old boy. At 2 3/12 years of age, the boy developed ataxia. One month later, a malignant cerebellar tumor was diagnosed by magnetic resonance tomography. The boy underwent a macroscopically complete resection. Histological diagnosis revealed medulloblastoma. Postoperative magnetic resonance scans showed no residual disease or spinal seedings and the patient was classified as T3 M0 according to Chang. Three weeks after surgery, chemotherapy with ifosfamide, VP 16, and methotrexate was started. One month later, a positive Babinski sign developed, computed tomography scans showed no abnormalities. Chemotherapy was continued with cisplatin plus cytosine arabinoside as well as cisplatin plus ifosfamide and methotrexate. After 4 months of chemotherapy, a seizure and weakness of the legs occurred. Magnetic resonance scans revealed massive meningeal seeding and 400 tumor cells/microliters were found in the cerebrospinal fluid. Craniospinal irradiation was initiated, and the neurologic symptoms resolved. The boy died, however, 4 months later due to progressive spinal disease. CONCLUSIONS: The early and rapid meningeal progression during chemotherapy is uncommon. The case demonstrates the difficulties of diagnosis and differential diagnosis.

Antineoplastic Combined Chemotherapy Protocols↗

[Boeck's disease with spinal involvement and retroperitoneal fibrosis].

Sarcoidosis (Morbus Boeck) is a frequently found disease of the reticulohistiocytic system. Etiology and pathogenesis were mostly unknown until today. Histologic study of sarcoidosis shown granuloma with epitheloid cells similar to tuberculosis, often with hyalinization but without cheesy necrosis. The disease mostly starts with a mediastinal and cervical lymphadenopathy. Radiographically, the disease shows three typical stages when spreading to the lungs. Bone involvement in lymphomas, especially vertebral involvement, is found to be rare. A case with an additional retroperitoneal fibrosis is reported from our own hospital.

Aged↗

[Changes in bladder contractility in spinal cord diseases].

Changes in bladder contractility were studied by repeated cystomanometry in 147 patients suffering from central medullary lesions of sudden onset, including 93 cases of spinal shock. The morphology of contraction was always modified in the same fashion though intermediate stages were apparent, some being quite characteristic of neurological dysfunction. This process, which ends in a phasic contraction reproduces the stages of ontogenesis. When contractions are present, but do not produce complete bladder emptying, spontaneous improvement is unlikely in the presence of a complete lesion; sphincterotomy should therefore be considered at an early stage.

Adolescent↗

Dissociated loss of vibration, joint position and discriminatory tactile senses in disease of spinal cord and brain.

The clinical functions of the posterior columns of the spinal cord and the signs of disease of these structures have been debated for years. Todd in 1847 and Schiff in 1858 knew the functions of the posterior columns and 10 years later Brown-Séquard knew as well. Reynolds, Romberg, and Duchenne, each described a posterior column syndrome based on a disease in which the primary lesion was not in the posterior columns. In the last 150 years almost every white matter structure of the cord has been credited with serving the sensations that we now know are a function of the posterior columns. Vibration, joint position and movement as well as discriminatory touch each seem to be served by separate fibres of the posterior columns and medial lemniscus. There is evidence of this in cat and man. These sensations may be lost individually, totally, or in certain stereotyped combinations. Vibration or joint sense is commonly lost alone. When a discriminatory touch sensation is lost with one other sense, it is almost inevitably joint position sense. Absent discriminatory touch and vibration sense with normal joint position sense appears to be unknown. This functional separation continues into the thalamus. At the highest level there is no evidence that vibration sense has any conscious somatosensory cortical affiliation, while joint position and discriminatory touch senses definitely do.

Brain↗

Congenital, hereditary and developmental spinal cord disease.

A number of papers have been published during 1991 in the area of congenital, hereditary and developmental spinal cord disorders, which discuss a wide range of rare spinal anomalies; there have, however, been no significant advances. The diagnosis of these disorders has been enhanced by the use of magnetic resonance imaging (MRI).

Cauda Equina↗