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MRI Brain Imaging Patterns in Acute Intermittent Porphyria: A Retrospective Observational Study with
Prateek Malik1, Pavithra Mannam2, Manas Sharma1
1From the Division of Neuroradiology (P.M., M.S., S.P.), Department of Medical Imaging, London Health Sciences Centre, Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Department of Radiodiagnosis (P.M., R.S.N., B.T.S.), Neurological Sciences (A.S.), Christian Medical College, Vellore, Tamil Nadu, India and Department of Radiology (S.M.), Jerudong Park Medical Centre, Bandar Seri Begawan, Brunei.
Background And Purpose:
The CNS imaging manifestations of acute intermittent porphyria (AIP) are poorly systematized. We aimed to characterize the spectrum of MRI brain findings and evaluate the association between dysautonomia, CNS findings, and enteric manifestations.
Materials And Methods:
We retrospectively identified patients with biochemically confirmed AIP who presented between 2008 and 2018 with acute neurovisceral episodes. This observational study did not include a control group. MRI brain studies performed during acute presentations were reviewed at the study level and categorized by predominant anatomical distribution into lobar-cortical, hippocampal, cortical-subcortical, deep gray matter, and normal patterns. Clinical and biochemical parameters were analyzed at the episode level with respect to dysautonomia status.
Results:
Twenty-three patients with 27 acute neurovisceral episodes were identified; 17 underwent MRI, yielding 28 studies. The lobar-cortical pattern was most frequent (9/28; 32.1%), followed by hippocampal (6/28; 21.4%), cortical-subcortical (3/28; 10.7%), and deep gray matter (3/28; 10.7%); 7 of 28 studies (25.0%) were normal. Two patients showed evolving findings across serial MRI. Dysautonomia, documented in 12/27 (44.4%) episodes, was significantly associated with ileus (p < 0.001) and hyponatremia (p = 0.02).
Conclusions:
AIP is associated with a broader range of MRI brain appearances than classical PRES alone, with overlap from seizure-related, metabolic, and electrolyte-related processes; no single pattern is diagnostic on its own. The observed clustering of dysautonomia, ileus, and hyponatremia, together with the inverse correlation between aminolevulinic acid and serum sodium, supports a shared autonomic basis for these manifestations. Recognizing this combined imaging and clinical pattern may help raise AIP as a diagnostic consideration.
Insights
Acute intermittent porphyria (AIP) shows diverse brain MRI findings, not limited to PRES. Dysautonomia, ileus, and hyponatremia suggest a shared autonomic basis in AIP neurovisceral episodes.
Area of Science:
- Neurology
- Radiology
- Medical Genetics
Background:
- Acute intermittent porphyria (AIP) central nervous system (CNS) imaging findings are not well-defined.
- Previous studies have not systematically characterized AIP's MRI brain manifestations.
Purpose of the Study:
- To define the spectrum of MRI brain findings in acute intermittent porphyria (AIP).
- To investigate the relationship between autonomic dysfunction, CNS findings, and gastrointestinal symptoms in AIP.
Main Methods:
- Retrospective analysis of 27 neurovisceral episodes in 23 biochemically confirmed AIP patients (2008-2018).
- Review of 28 brain MRI studies performed during acute AIP episodes.
- Categorization of MRI findings by anatomical distribution: lobar-cortical, hippocampal, cortical-subcortical, deep gray matter, and normal.
- Analysis of clinical and biochemical data, including autonomic dysfunction status.
Main Results:
- The lobar-cortical pattern was the most common MRI finding (32.1%), followed by hippocampal (21.4%).
- 25% of MRI studies were normal; some patients showed evolving changes on serial imaging.
- Autonomic dysfunction occurred in 44.4% of episodes and was significantly linked to ileus and hyponatremia.
Conclusions:
- AIP presents with varied MRI brain appearances, overlapping with PRES, seizure, metabolic, and electrolyte disturbances.
- The co-occurrence of autonomic dysfunction, ileus, and hyponatremia suggests a common autonomic pathway in AIP.
- Recognizing this pattern aids in considering AIP as a diagnosis.
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