Subacute Sclerosing Panencephalitis

Subacute sclerosing panencephalitis (SSPE) is chronic progressive encephalitis of childhood and young adoloscent due to persistent measles virus infection. This case illustrates a 14 year old girl presented with short history of intellectual decline, abnormal behavior, myoclonus and altered consciousness with suggestive neuroimaging mimicking metachromatic leucodystrophy. Subsequently she was diagnosed to be a case of Subacute sclerosing panencephalitis (SSPE) on the basis of Electroencephalography (EEG) and Cerebrospinal fluid(CSF) measles antibody titer. J Bangladesh Coll Phys Surg 2019; 37(4): 205-208

Arylsulfatase A: An Important Metabolic Factor in Pathophysiology of Different Diseases

Arylsulfatase A (ARSA) is a lysosomal enzyme that plays an important role in catalysis of degradation of cerebrosidesulphate. The deficiency of this lysosomal enzyme causes an autosomal recessive disorder, called metachromatic leucodystrophy. However, a low ARSA activity can be observed in clinically healthy people, called ARSA pseudodeficiency. In our study we investigated the possible linkage between ARSA activity and sulfatide deficiency causing characteristic aspects of degenerative diseases, such as end stage kidney disease, type 2 Diabetes mellitus, Parkinson syndrome, prostate cancer and HIV (Human Immunodeficiency Virus) infection. We used a spectrophotometric method to determine the activity of ARSA. This method of enzyme dosage is based on a 4 hour long hydrolysis of the ARSA enzyme on 4-nitrocatechol sulfate (p-NCS) substrate. The unit of this measurement is nmol/ml/4h. Our findings show significant values in type 2 diabetes, Parkinson syndrome and chronic kidney disease. The importance of sulfatide in these diseases is well-known, thus presumably the variation of the ARSA’s activity might play an important role in the pathophysiology of these diseases, involving a vicious cycle between sulfatide degradation andthese diseases.