Answer for CoW 02 April 2017
Findings
Rasmussen Encephalitis: Unilateral cortical atrophy with ex-vacuo ventricular dilatation
Discussion
Rasmussen encephalitis, sometimes referred to as Rasmussen syndrome, is a rare disorder of the central nervous system characterized by chronic progressive inflammation (encephalitis) of one cerebral hemisphere. As a result, the patient usually experiences frequent episodes of uncontrolled electrical disturbances in the brain that cause epileptic seizures (epilepsy), and progressive cerebral destruction. With time, further symptoms may include progressive weakness of one side of the body (hemiparesis), language problems (if on the left side of the brain) and intellectual disabilities. The exact cause of this disorder is not known. The two leading ideas are that the brain inflammation might be a reaction of a foreign antigen (infection) or an autoimmune disease limited to one side of the brain resulting in brain damage. It occurs mostly, but not always, in children between the ages of two and ten years, and in many patients the course of the disease is most severe during the first 8 to 12 months. After the peak inflammatory response is reached, the progression of this disorder appears to slow or stop and the patient is left with permanent neurological deficits.
Rasmussen Encephalitis: Unilateral cortical atrophy with ex-vacuo ventricular dilatation
Discussion
Rasmussen encephalitis, sometimes referred to as Rasmussen syndrome, is a rare disorder of the central nervous system characterized by chronic progressive inflammation (encephalitis) of one cerebral hemisphere. As a result, the patient usually experiences frequent episodes of uncontrolled electrical disturbances in the brain that cause epileptic seizures (epilepsy), and progressive cerebral destruction. With time, further symptoms may include progressive weakness of one side of the body (hemiparesis), language problems (if on the left side of the brain) and intellectual disabilities. The exact cause of this disorder is not known. The two leading ideas are that the brain inflammation might be a reaction of a foreign antigen (infection) or an autoimmune disease limited to one side of the brain resulting in brain damage. It occurs mostly, but not always, in children between the ages of two and ten years, and in many patients the course of the disease is most severe during the first 8 to 12 months. After the peak inflammatory response is reached, the progression of this disorder appears to slow or stop and the patient is left with permanent neurological deficits.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
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Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!