muramidase and Reflex--Abnormal

Lysozyme in tears is secreted from the main lacrimal gland innervated by a parasympathetic nerve via the greater petrosal nerve. Lysozyme activity in tears was analysed in 10 cases of unilateral facial paralysis due to the cerebellopontine angle (CPA) tumor and 10 normal cases. The lysozyme concentration in tears on the paralysed side collected by Schirmer No. 2 test was significantly lower than that on the healthy side, or bilateral sides of normal subjects. This decreased concentration on the paralysed side is due to the interruption of the reflex are at the level of CPA. It is concluded that our new method is more useful for topographical diagnosis of facial paralysis than is the conventional Schirmer's filter paper test.

Topics: Adult; Cerebellar Neoplasms; Cerebellopontine Angle; Clinical Enzyme Tests; Facial Paralysis; Humans; Middle Aged; Muramidase; Reflex, Abnormal; Tears

An 18-year-old boy showed childhood onset of mental retardation, neurogenic muscle atrophy with hyperreflexia, Marfan-like features, multiple epiphyseal dysplasia, increased urinary excretion of dermatan sulfate, and decreased lysosomal enzyme activities in beta-galactosidase, beta-glucuronidase, and N-acetyl-beta-D-glucosaminidase. This case may be a new syndrome, the combination of neurogenic muscle atrophy with lysosomal enzyme deficiencies.

Topics: Adolescent; Diagnosis, Differential; Humans; Intellectual Disability; Leukocytes; Male; Metabolism, Inborn Errors; Mucopolysaccharidoses; Muramidase; Muscular Atrophy; Reflex, Abnormal; Syndrome