Acquired cerebral hemiatrophy: idiopathic?

Area of Science:

• Neurology
• Neuroscience
• Medical Case Reports

Background:

• Acquired cerebral hemiatrophy is a rare neurological condition characterized by progressive unilateral brain atrophy.
• Etiologies are diverse, often involving vascular events, infections, or inflammatory processes.
• Rasmussen's encephalitis is a known cause, typically presenting with intractable epilepsy and cognitive decline.

Observation:

• A young male patient presented with initially progressive, then stabilizing, acquired cerebral hemiatrophy.
• The patient experienced no seizures during the illness.
• Extensive investigations failed to reveal a definitive cause, but cerebrospinal fluid analysis showed a mild pleocytosis during the progressive phase.

Findings:

• The clinical course and findings suggest an underlying unidentified infective or inflammatory process.
• The case challenges typical presentations of Rasmussen's encephalitis, suggesting it may represent a spectrum of disease.
• Multimodality imaging proved crucial in the diagnostic evaluation of cerebral hemiatrophy.

Implications:

• This case expands the potential spectrum of Rasmussen's encephalitis beyond typical epileptic presentations.
• Highlights the importance of considering inflammatory/infectious etiologies even without seizures in cerebral hemiatrophy.
• Underscores the value of advanced neuroimaging techniques in diagnosing complex neurological conditions.