Sushant Agarwal

STUDY OF CHIARI MALFORMATIONS IN A TERTIARY CARE HOSPITAL IN NORTH-EAST INDIA

Address for Correspondence: Dr. Pradipta Ray Choudhury, Assistant Professor, Department of Anatomy, Silchar Medical College and Hospital, Silchar-788014, Assam, India. E-Mail: prabahitabaruah@gmail.com Background: Chiari malformations are herniation of part of cerebellum into the foramen magnum. Professor Hans Chiari classified chiari malformations into four types: type I to type IV. Type I chiari malformation is the most extensively studied entity. Type III and IV are very rare variety. Aim: Magnetic Resonance Imaging (MRI) study of the different types of chiari malformations in a tertiary care hospital in North-East India. Materials and Methods: After performing MRI of brain and spinal cord, 63 cases of chiari malformations were included in the study. The cases were classified according to the classification of Hans Chiari. Results: 73.02% cases were type I followed by 22.22% cases of type II chiari malformations. Male predominated female and more than half of the patients were under twenty years of age. Hydrocephalus and syringomyelia were mostly associated with type I chiari malformations. Conclusion: various aspects of such a rare type of congenital anomaly were studied in this part of the country.

MULTI SYSTEM ATROPHY: REPORT OF TWO CASES

Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, clinically characterized by Parkinsonian, autonomic, cerebellar and pyramidal signs. We describe two patients showing different presentations of the same disease. The patient on case 1 presents features of MSA-C or olivopontocerebellar atrophy with the pontine “Cross sign” on brain MRI. The second case reports a patient presenting MSA-P or striatonigral degeneration and the brain MRI shows lenticular nucleus sign alteration. Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, characterized by several combinations of parkinsonian, autonomic, cerebellar and pyramidal signs.1-5. The incidence is 0.6 cases/100000/year and the prevalence ranges from 1.86 to 4.9 cases/1000001. The term MSA defines a distinct clinicopathological entity, including olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome, which have been described as different diseases for many years and still lead to terminology confusion. The actual consensus tries to define the diagnostic criterions and recommends the term MSA-P when parkinsonian features predominate, and the term MSA-C when cerebellar signs rules the clinical picture. There is some doubt if the term Shy-Drager syndrome should be substitute, as most cases of MSA patients develop autonomic dysfunction during their course.6 Horimoto et al.7 believe that some patients present a distinct form of MSA in which dysautonomic symptoms predominate, considering the utility to classify that form as MSA-A. Recently, neuroimaging studies, especially the MRI, have showed some alterations that, although not specific, may help the diagnostic of different forms of MSA. We report two MSA cases with different clinical features and their characteristic MRI alterations.