Nose to back: compatibility of nasal chondrocytes with environmental conditions mimicking a degenerated intervertebral disc.

Nasal chondrocytes (NCs) have gained increased recognition for cartilage tissue regeneration. To assess NCs as a source for cell therapy treatment of intervertebral disc (IVD) degeneration, tissue-forming properties of NCs under physiological conditions mimicking the degenerated IVD were compared to those of mesenchymal stromal cells (MSCs) and articular chondrocytes (ACs), two cell sources presently used in clinical trials. Cells were cultured in a combination of low glucose, hypoxia, acidity and inflammation for 28 d. Depending on the conditions, cells were either cultured in the absence of instructive growth factors or underwent chondrogenic instructional priming by addition of transforming growth factor ß1 (TGFß1) for the first 7 d. Histology, immunohistochemistry, biochemistry, enzyme-linked immunosorbent assay (ELISA) and quantitative real-time reverse transcriptase-polymerase chain reaction (qRT-PCR) analyses demonstrated limited cell maintenance and accumulation of cartilaginous extracellular matrix for MSCs in IVD conditions. ACs maintained a steady accumulation of glycosaminoglycans (GAGs) throughout all non-acidic conditions, with and without priming, but could not synthesise type II collagen (Col2). NCs accumulated both GAGs and Col2 in all non-acidic conditions, independent of priming, whereas MSCs strongly diminished their GAG and Col2 accumulation in an inflamed environment. Supplementation with inflammatory cytokines or an acidic environment affected NCs to a lower extent than ACs or MSCs. The data, overall indicating that in an inflamed IVD environment NCs were superior to ACs and MSCs, encourage further assessment of NCs for treatment of degenerative disc disease.

Chronic spinal cord stimulation in medically intractable orthostatic tremor.

BACKGROUND: Orthostatic tremor with its sense of unsteadiness when standing may have a devastating effect on affected persons. Currently, there are no other treatment options in those who do not respond or who do not tolerate medical treatment. OBJECTIVES: To report on a pilot study on spinal cord stimulation in medically intractable orthostatic tremor. METHODS: Chronic spinal cord stimulation (SCS) was performed in two patients with medically-intractable orthostatic tremor via quadripolar plate electrodes implanted at the lower thoracic spine. The electrodes were connected to implantable pulse generators. RESULTS: Subjective and objective improvement of unsteadiness was achieved within a frequency range of 50 to 150 Hz, and occurred in the presence of stimulation-induced paraesthesia. With optimized stimulation settings polygraphic electromyelogram (EMG) recordings continued to show the typical 14-16 Hz EMG activity. The beneficial effect of SCS was maintained at long-term follow-up. CONCLUSIONS: The results of this pilot study indicate that SCS may be an option in patients with otherwise intractable orthostatic tremor.

Spontaneous craniocervical osseous fusion resulting from cervical dystonia. Case report.

The authors report on a 31-year-old man with spontaneous craniocervical osseous fusion secondary to cervical dystonia (CD). After an 8-year history of severe CD, the patient developed a fixed rotation of his head to the right. Three-dimensional computerized tomography reconstructions revealed rotation and fixation of the occiput and C-1 relative to C-2, which was similar to that seen in atlantoaxial rotatory fixation. There was abnormal ossification of the odontoid facet joints and ligaments. Additional ossification was observed in the cervical soft tissue bridging the lateral mass of C-1 and the occiput. The patient underwent partial myectomy of the dystonic left sternocleidomastoid muscle and selective posterior ramisectomy of the right posterior neck muscles; postoperatively he experienced relief of his neck pain. In patients with CD refractory to conservative treatment, the appropriate timing of surgical treatment is important.

A prolonged outbreak of Shigella sonnei infections in traditionally observant Jewish communities in North America caused by a molecularly distinct bacterial subtype.

During 1994-1996, Shigella sonnei outbreaks occurred in 8 North American traditionally observant Jewish communities. These communities remain relatively separate from neighboring populations while maintaining close contact by travel with coreligionists in other cities. Epidemiologic investigations suggested community-to-community transmission via travel. Outbreak-related and control isolates of S. sonnei from each city were subtyped by pulsed-field gel electrophoresis (PFGE) to confirm an epidemiologic linkage between outbreaks. Forty-three (94%) of 46 outbreak-related isolates had closely related PFGE patterns, constituting a single subtype; 33 (94%) of 35 control isolates demonstrated unrelated PFGE patterns. Several patterns differing by < or = 3 bands were identified within the outbreak subtype; one of these accounted for 65% of outbreak isolates. Hence, a single subtype of S. sonnei caused an international outbreak involving 8 traditionally observant Jewish communities, but not neighboring populations, over a 2-year period, suggesting sustained propagation of the epidemic strain between communities.

[Adults with hereditary fructose intolerance: risks of fructose infusion]. / Erwachsene mit hereditärer Fructoseintoleranz: Gefährdung durch Fructoseinfusion.

After her first grand mal seizure a 30-year-old woman was given a fructose infusion by an emergency doctor. On admission to hospital she complained of severe nausea. Ultrasonography revealed hepatosplenomegaly and the gamma-GT concentration was raised to 25 U/l. As hyperinsulinism was suspected an oral glucose tolerance test was suggested, but refused by the patient. She reported marked aversion to all sweet foods. Examination of an endoscopically obtained liver biopsy revealed clear reduction in fructoaldolase activity in liver tissue, i.e. the diagnosis of hereditary fructose intolerance. Three of the patient's siblings were also affected. The widespread use of infusion solutions containing sorbitol and fructose has twice proved acutely hazardous in this patient and is generally life-threatening for persons with an inborn error of metabolism whose pathologic status often remains undiagnosed to an adult age.

[The clinical picture and course in basilar impression]. / Klinisches Bild und Verlauf bei basilärer Impression.

We have reviewed 29 cases of patients who suffered from basilar impression and had undergone hospital treatment since 1969. 12 of these patients were reexamined. The predominant symptoms were lesions of the long tracts while the ,,classical signs" such as headache and nystagmus occurred less frequently than expected. Only a quarter of our patients showed a marked deterioration during follow-up. The outcome for four patients who underwent operation is reported.

[Value of visual evoked potentials (VEP) in compression of the anterior visual pathway, especially in the area of the chiasm]. / Die Wertigkeit der visuell evozierten Potentiale (VEP) bei Kompression der vorderen Sehbahn, speziell in der Chiasmaregion.

We investigated 86 patients by pattern half-field stimulation presenting clinically with temporal field-defects or with a mass causing compression of the visual pathways found by C.T.-scanning. Pattern half-field stimulation proved to be a very sensitive method for detecting a compression of the anterior visual pathways. In 65 cases the results of perimetry and VEP were congruent, 6 times the evoked potential was pathologic the perimetry showing no abnormalities, only 1 false negative VEP was observed. Thus we stress the importance of an investigation by VEP including half-field stimulation in all patients with suspected compression of the anterior visual pathways.

[EMG and evoked potentials in adrenomyeloneuropathy]. / EMG und evozierte Potentiale bei der Adrenomyeloneuropathie.

In a 31 year old patient with adrenocortical gland insufficiency and minor clinical signs of involvement of peripheral and central nervous system an adrenoleucodystrophy was diagnosed, based upon the characteristic elevation of hexacosanoic acid in plasma. The motor and sensory nerve conduction velocities in upper and lower extremities were markedly reduced; in accordance, the somatosensory evoked potentials showed distinct reduction in the peripheral part. In contrast, the interpeak latencies N 20 - NSEP 3 (median nerve) and N 22 - P 40 (tibial nerve) were in the upper normal range, so that a damage of the central sensory system could not be evaluated. The visual and brainstem acoustic evoked potentials were a little prolonged. The problems in differential diagnosis of this form of adrenoleucodystrophy, which especially involves the peripheral nervous system, are discussed.

[Cerebral angiography in transitory global amnesia]. / Zerebrale Angiographie bei transitorischer globaler Amnesie.

33 patients with transient global amnesia were examined by cerebral angiography. The presence of arterial lesions correlated with neurological symptoms, as well as with amnesia. In 55% of cases no pathological alterations were found, so that the pathogenesis of the transient global amnesia remained unclear. For clinical purposes, cerebral angiography is recommended only in those cases which show other neurological symptoms besides the transient global amnesia.