Infantile Alexander Disease: Case Report and Review of Literature
Published: June 1, 2017 | DOI: https://doi.org/10.7860/JCDR/2017/26875.10106
Soumyabrata Sarkar, Rupam Sinha, Amitabha Chakraborty, Tanya Khaitan, Biyas Bhowmik
1. Reader, Department of Oral Medicine and Radiology, Haldia Institute of Dental sciences and Research, Haldia, West Bengal, India
2. Professor and Head, Department of Oral Medicine and Radiology, Haldia Institute of Dental sciences and Research, Haldia, West Bengal, India
3. Professor and Head, Department of Pedodontics and Preventive Dentistry, Haldia Institute of Dental sciences and Research, Haldia, West Bengal, India
4. Clinical Tutor, Department of Dentistry, Murshidabad Medical College and Hospital, Berhampore, West Bengal, India
5. Senior Lecturer, Department of Oral Medicine and Radiology, Haldia Institute of Dental sciences and Research, Haldia, West Bengal, India
Correspondence Address :
Dr. Soumyabrata Sarkar,
Reader, Department of Oral Medicine and Radiology, Haldia Institute of Dental sciences and Research,
Haldia, West Bengal –721645, India.
E-mail: dr.rupsarkar@gmail.com
Abstract
Alexander Disease (AD) is an autosomal dominant leukodystrophy and occurs predominantly in infants and children. It usually results in death within ten years after onset. Among the four subtypes, infantile form comprises the most of affected individuals. It presents in the first two years of life, typically with progressive psychomotor deficiency, loss of developmental milestones, seizures, and pyramidal signs. Clinical and magnetic resonance image findings usually establish diagnosis of AD. Here, we present a case of Infantile AD with characteristic clinical and radiological features.
Keywords
Leukodystrophy, Magnetic resonance imaging, Seizures