Rapidly Progressive Dementia Associated with ASIA Syndrome
Carlos Guevara Oliva1, Rodrigo Naves1
1School of Medicine, University of Chile
Objective:
N/A
Background:
Autoimmune/Inflammatory Syndrome Induced by Adjuvants (ASIA syndrome) is a condition triggered by exposure to adjuvants.
Design/Methods:

A 33-year-old woman, previously self-sufficient in activities of daily living, presented with a two-month history of daily episodes involuntary movements involving the head, trunk, and lower limbs,  consistent with  myoclonic jerks (Video 1).   The episodes were associated with partial loss of consciousness. She also reported mild to moderate polyarthralgia, treated symptomatically with intravenous hydrocortisone.  In addition to the above, her husband noted progressive cognitive and functional decline. 

Her medical history included polycystic ovary, insulin resistance, use of combined  oral contraceptives (drospirenone and ethinylestradiol), bilateral silicone breast implants, and smoking. Her family history included epilepsy in her mother and two brothers


Results:

 

On admission, neurological  examination revealed disorientation to date, significantly impaired short-term recall, visuospatial memory impairment, and limited attention span. Speech was fluent  with frequent paraphasic errors and anomia. She had mild gait ataxia.

A rapidly progressive dementia was suspected. Studies ruled out autoimmune diseases and thyroid disfunction. Brain MRI with contrast and sleep-deprived EEG showed no abnormalities.  Cerebrospinal fluid analysis was normal. Genetic studies for prion protein and  Huntington's chorea were normal. Copper in urine and blood was normal. As part of a paraneoplastic study, breast MRI revealed signs of intracapsular rupture of both silicone breast implants (Figure 1).

The implants were surgically removed with surgical cleaning of the silicone capsules. The patient's symptoms completely remitted within 7 days.

Based on these findings and the neurological and systemic symptoms, a diagnosis of ASIA syndrome was considered.

 

Conclusions:

This case emphasizes the need to consider ASIA syndrome in patients presenting with unexplained cognitive decline and seizures, particularly in the context of silicone breast implants. Remarkably, studies have reported that 50-98% of patients experienced improvement of silicone-related complaints after explantation.

10.1212/WNL.0000000000216143
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