ABSTRACT
Symptomatic treatment of stiff-person syndrome (SPS) might be challenging and a significant improvement of stiffness and rigidity is generally reached with high doses of benzodiazepines or baclofen causing side effects. A 71-year old woman diagnosed with SPS complained of marked stiffness of trunk and lower limb muscles with sudden painful spasms. She was unable to walk and she could not lean on her right leg. Cortical silent period (CSP) duration evaluated from right abductor pollicis brevis (APB) with transcranial magnetic stimulation was shortened. Polygraphic electromyographic (EMG) evaluation from paraspinal and leg muscles disclosed continuous motor unit activity at rest with interference muscular pattern. Symptomatic treatment with diazepam was withdrawn because of excessive sedation. In order to relieve the intense lumbar pain, she was prescribed pregabalin; since the day after, rigidity and painful spasms dramatically improved and she could walk without assistance. The clinical benefit persisted at 3 months follow-up and was paralleled by almost complete disappearance of EMG activity at rest and prolongation of CSP. The clinical and electrophysiological data in this SPS patient suggest the possible efficacy of pregabalin as symptomatic treatment without any significant side effects, which needs to be replicated in larger case series.
Subject(s)
Analgesics/therapeutic use , Stiff-Person Syndrome/drug therapy , gamma-Aminobutyric Acid/analogs & derivatives , Aged , Back Pain/drug therapy , Back Pain/etiology , Electroencephalography , Electromyography , Female , Glutamate Decarboxylase/immunology , Humans , Muscle, Skeletal/physiopathology , Neurologic Examination , Pregabalin , Stiff-Person Syndrome/complications , Stiff-Person Syndrome/physiopathology , Transcranial Magnetic Stimulation , gamma-Aminobutyric Acid/therapeutic useABSTRACT
Endothelial intercellular adhesion molecule-1 (ICAM-1) and glycoprotein E-selectin (ELAM-1) allow the homing of leukocytes to inflammation sites. A circulating form of ICAM-1 markedly increases in inflammatory CNS disorders. In the present study, the serum levels of ICAM-1, ELAM-1 and tumor necrosis factor-alpha (TNF-alpha) were measured in patients with acute (AIDP) and chronic (CIDP) inflammatory demyelinating polyneuropathies and cryoglobulinemic neuropathy (CGN). Immunoenzymometric assays revealed increased sICAM-1 levels in some of these patients; furthermore, high titres of ELAM-1 and TNF-alpha were detected in two patients with AIDP and one patient with CGN. Our data extend previous observations on inflammatory PNS disorders by showing that, in addition to ICAM-1, ELAM-1 also represents a useful marker of endothelial activation and that, taken together, the two molecules may serve as an indicator of specific pathogenetic mechanisms.
Subject(s)
Cell Adhesion Molecules/blood , Intercellular Adhesion Molecule-1/blood , Membrane Glycoproteins/blood , Neuritis/blood , Peripheral Nervous System Diseases/blood , Tumor Necrosis Factor-alpha/metabolism , Adult , Aged , Cryoglobulinemia/blood , Demyelinating Diseases/blood , E-Selectin , Enzyme-Linked Immunosorbent Assay , Female , Humans , Male , Middle Aged , Multiple Sclerosis/blood , Polyneuropathies/bloodSubject(s)
Erythrocytes/analysis , Lipoproteins/deficiency , Vitamin B 12/blood , Animal Feed , Animals , Kidney/pathology , Male , Necrosis , Organ Size , Rats , Rats, Inbred StrainsABSTRACT
El objetivo de este trabajo fue medir la concentración de vitamina B12 en plasma y en eritrocitos de ratas alimentadas con una dieta hipolipotrópica. No conocemos que estos datos experimentales en ratas colina deficientes hayan sido reportados previamente. Se utilizaron ratas Wistar machos recién destetadas que se separaron en 4 grupos: CD, alimentadas con dieta hipolipotrópica; CS, alimentadas con dieta similar pero suplementada con colina e individual y diariamente apareadas con ratas líderes de grupo CD; C, alimentadas con dieta comercial, y W, que fueron sacrificadas en el momento del destete. Los cambios morfológicos en hígado, riñón y sangre periférica fueron correlacionados con la concentración de vitamina B12 en sangre determinada en los días 0 (grupo W) 5§ y 8§ (CD y CS). Los cambios hepáticos, renales y de sangre periférica fueron similares a los previamente publicados. En los animales del grupo CD se encontró anemia con marcadas alteraciones eritrocitarias y trombocitopenia, además de hígado graso y necrosis renal. Una disminución significativa en la concentración de vitamina B12 en plasma y eritrocitos en el 8§ día se encontró en los grupos CD y CS. La suplementación de colina previno el daño renal y hepático pero no así los cambios en sangre periférica debidos a la deficiencia de vitamina B12. El grupo CS mostró una menor concentración de vitamina B12 que el grupo CD con necrosis renal, lo que permite suponer una liberación por parte del tejido renal necrosado
Subject(s)
Rats , Animals , Male , Diet , Erythrocytes/analysis , Lipoproteins/deficiency , Vitamin B 12/blood , Body Weight , Kidney/pathology , Necrosis , Organ Size , Rats, Inbred StrainsABSTRACT
El objetivo de este trabajo fue medir la concentración de vitamina B12 en plasma y en eritrocitos de ratas alimentadas con una dieta hipolipotrópica. No conocemos que estos datos experimentales en ratas colina deficientes hayan sido reportados previamente. Se utilizaron ratas Wistar machos recién destetadas que se separaron en 4 grupos: CD, alimentadas con dieta hipolipotrópica; CS, alimentadas con dieta similar pero suplementada con colina e individual y diariamente apareadas con ratas líderes de grupo CD; C, alimentadas con dieta comercial, y W, que fueron sacrificadas en el momento del destete. Los cambios morfológicos en hígado, riñón y sangre periférica fueron correlacionados con la concentración de vitamina B12 en sangre determinada en los días 0 (grupo W) 5º y 8º (CD y CS). Los cambios hepáticos, renales y de sangre periférica fueron similares a los previamente publicados. En los animales del grupo CD se encontró anemia con marcadas alteraciones eritrocitarias y trombocitopenia, además de hígado graso y necrosis renal. Una disminución significativa en la concentración de vitamina B12 en plasma y eritrocitos en el 8º día se encontró en los grupos CD y CS. La suplementación de colina previno el daño renal y hepático pero no así los cambios en sangre periférica debidos a la deficiencia de vitamina B12. El grupo CS mostró una menor concentración de vitamina B12 que el grupo CD con necrosis renal, lo que permite suponer una liberación por parte del tejido renal necrosado (AU)
Subject(s)
Rats , Animals , Male , Vitamin B 12/blood , Lipoproteins/deficiency , Diet , Erythrocytes/analysis , Kidney/pathology , Necrosis , Organ Size , Body Weight , Rats, Inbred StrainsSubject(s)
Acquired Immunodeficiency Syndrome/diagnosis , Homosexuality , Sarcoma, Kaposi/diagnosis , Adult , Argentina , Humans , MaleABSTRACT
Weanling male rats fed on a hypolipotropic diet develop acute renal failure whose morphological features vary from focal tubular necrosis to cortical necrosis. We have sequentially studied the hemostatic mechanism in correlation with the morphology of various tissues, mainly renal and hepatic, in choline-deficient rats as well as in three control groups. No important changes were observed in the hemostatic mechanisms before the development of tubular necrosis. Along with tubular necrosis a consumption coagulopathy was found, evidenced mainly by a decrease in the activity of factors V and VIII as well as a prolongation in PTTK and Quick's time and a decrease in platelets. Fibrin degradation products were found in serum and urine and soluble fibrin monomer complexes in the former. Following tubular necrosis thrombi were found in the renal microvasculature. It is possible to speculate that the tubular necrosis induced by choline deficiency could produce an activation of the coagulation system which in turn would lead to thrombosis of the renal microcirculation and cortical necrosis.
