ABSTRACT
Objective To investigate the clinical and pathological features of Uurich congenital muscular dystrophy (UCMD). Methods The clinical aspects of 3 patients with UCMD, 2 with Duchenne muscular dystrophy (DMD) and 1 with congenital muscular dystrophy 1A (MDC1A) were analyzed. And the muscle specimens from these patients were studied using immunohistochemistry and immunofluorescence staining. Results UCMD was clinically characterized by neonatal hypotonia with proximal contracturos and distal hyperlaxity at birth or early infancy. Histochemical staining revealed muscle frber hypoplasia andinterstitium proliferation. Immunohistochemistry staining with anti-collagen Ⅵ antibody revealed complete(1/3) or partial (2/3) deficiency of collagen Ⅵ in the sarcolemma and interstitial matrix. Partial deficiency was better demonstrated by immunofluorescence staining. Conclusions The proximal contractures and distal hyperlaxity is the clinical hallmark of UCMD. Collagen Ⅵ immunolabelling can confirm the diagnosis of UCMD.
ABSTRACT
Purpose To investigate the influence of paraquat on substantial nigra and doparine levels ofstriatum in C57BL mice. Methods 39 neonatal C57BL mice were randomly divided into 5 groups andwere given paraquat or 1-methyl-4-phenyl-1,2,3,6-tetra-hydropyridine(MPTP) orally in 10 th and 11 thdays odl; ( 1 ) MPTP 0.3 mg/kg, n = 8; (2) MPTP 20 mg/kg, n = 8; (3) paraquat 0.07 mg/kg, n = 8; ( 4 )paraquat 0.36 mg/kg, n = 8; (5) normal saline, n = 7. Adult spontaneous motor activity was observed atages of 120 days, then the mice were decapitated and the contents of dopamine(DA), serotonin(5-HT), andtheir metabolites in striatum were analyzed. Meanwhile, the dopamine neuons at the mesencephalon vereobserved by the method of ABC immunohistochemistry. Results Mice given Paraquat 0.36 mg/kg andMPTP 20 rng/kg showed a marked bypoactive behavior and reduced the striatal contents of DA andmetabolites without affecting 5-HT. Immunohistochemical staining showed that the amount of dopamineneurons at the midbrain decreased. Conclusions C57BL mice exposed to great amount of paraquat duringthe neonatal period could yield the alterations of behavior and some pathological and biochemical changessimilar to parkinson disease.