Early progression of brain atrophy in patients with anti-N-methyl-D-aspartate receptor encephalitis: Case reports
Hiroshi Kataoka1, Nobuhiro Sawa, Yasuyo Tonomura
1Department of Neurology, Nara Medical University, Kashihara, Nara, Japan.
Background:
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis responds to immunnotherapy, and approximately 80% of patients with this disorder fully recover or have only minor sequelae. Brain magnetic resonance imaging (MRI) does not show a specific abnormality, but some patients have progressive cerebral atrophy. The cerebral atrophy can become reversible after clinical improvement.
Methods:
We describe 3 patients with diffuse cerebral atrophy (DCA) on serial brain MRI.
Results:
Two women had the typical spectrum of this disorder, and one man had mainly psychiatric symptoms. In a woman with an ovarian tumor, DCA was reversible and DCA developed within about a half month. In another woman without a tumor, DCA was evident within 19 days and had progressed over the course of 4 years. The titers of anti-NMDAR antibodies in serum and cerebrospinal fluid (CSF) initially decreased, and low titers of the antibodies persisted. In a man without a tumor, DCA progressed within 14 days, and during this short period, he did not receive prolonged treatment with corticosteroids, various antiepileptic agents, or propofol, and he was free of seizures and ventilatory support.
Conclusion:
Not only a woman but also a man with anti-NMDAR encephalitis can have DCA in the early phase of this disorder. However, DCA can be reversible after clinical improvements. The early progression of DCA is not necessarily a poor prognostic factor.
Insights
Diffuse cerebral atrophy (DCA) can occur in anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis in both men and women. This early-stage brain atrophy may reverse with treatment, indicating it is not a poor prognostic sign.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is an autoimmune disorder typically responding to immunotherapy, with most patients achieving good recovery.
- While brain MRI often lacks specific findings, some patients develop progressive cerebral atrophy, which can be reversible.
Observation:
- This study reports on three patients (two women, one man) with anti-NMDAR encephalitis who exhibited diffuse cerebral atrophy (DCA) on serial brain MRI.
- DCA was observed in a patient with an ovarian tumor and was reversible; in another woman and a man without tumors, DCA progressed over varying timeframes.
Findings:
- The study highlights that DCA can manifest in the early phase of anti-NMDAR encephalitis in both sexes, including men presenting with predominantly psychiatric symptoms.
- Antibody titers in serum and cerebrospinal fluid (CSF) decreased during treatment, with low levels persisting.
- Rapid DCA progression in a male patient occurred without prolonged corticosteroid, antiepileptic, or propofol treatment, and he remained seizure-free.
Implications:
- Diffuse cerebral atrophy in the early stages of anti-NMDAR encephalitis is not exclusive to women and can occur even without specific treatments.
- The reversibility of DCA after clinical improvement suggests that early progression is not necessarily indicative of a poor long-term prognosis.
- These findings may help refine the understanding and prognostic evaluation of patients with anti-NMDAR encephalitis and associated cerebral atrophy.
More Related Videos
Related Concept Videos
01:19Encephalitis l: Introduction
01:26Encephalitis ll: Pathophysiology
01:26Brain Abscess l: Introduction
01:29Alzheimer Disease l: Introduction
01:23Alzheimer Disease ll: Pathophysiology