Authors :
Presenting Author: Xiaoyang Li, MD – Mayo Clinic, Rochester
Pranjal Gupta, MD – Department of Neurology – Mayo Clinic, Rochester; Kelsey Smith, MD – Department of Neurology – Mayo Clinic, Rochester; Ajay Madhavan, MD – Department of Radiology – Mayo Clinic, Rochester; Mohamed Rezk, MD – Department of Neurology – Mayo Clinic, Rochester; Anastasia Zekeridou, MD – Department of Neurology, Department of Laboratory Medicine and Pathology – Mayo Clinic, Rochester; Eoin Flanagan, MD – Department of Neurology, Department of Laboratory Medicine and Pathology – Mayo Clinic, Rochester; Andrew McKeon, MD – Department of Neurology, Department of Laboratory Medicine and Pathology – Mayo Clinic, Rochester; Sean Pittock, MD – Department of Neurology, Department of Laboratory Medicine and Pathology – Mayo Clinic, Rochester; Jeffrey Britton, MD – Department of Neurology – Mayo Clinic, Rochester; Divyanshu Dubey, MD – Department of Neurology, Department of Laboratory Medicine and Pathology – Mayo Clinic, Rochester
Rationale:
Faciobrachial dystonic seizure is a pathognomonic feature among patients with leucine-rich glioma-inactivated 1 (LGI1)-IgG autoimmune encephalitis. However, descriptions of lower extremity involvement are limited. Gait impairment and fall risks in LGI1 IgG autoimmune encephalitis are under recognized in clinical practice yet may lead to worse outcomes. This study aims to describe the prevalence, causes, and consequences of seizure-related falls and gait disturbances in LGI1-IgG autoimmune encephalitis.
Methods:
We retrospectively reviewed patients seen at our institution between January 1, 2000 and December 31, 2022. A total of 136 patients who fulfilled the following criteria were identified: (1) serum positivity for LGI1-IgG; (2) clinical phenotypes compatible with LGI1-IgG autoimmune encephalitis; (3) falls or near falls related to seizures. The clinical documentation, Magnetic Resonance Imaging (MRI), and electroencephalogram (EEG) data were collected and reviewed.
Results:
In this cohort of 136 patients, 27% (n=36) had falls or near falls related to seizures. Among those 36 patients, 23 (64%) were male. The median age was 67 years (range 49-86 years). Facio-brachio-crural (face-arm-leg) dystonic seizures (21/36, 58%) and drop attacks (9/36, 25%) were the most common causes of falls or near falls, followed by brachio-crural seizures (3/36, 8%), crural seizures (2/36, 6%) and focal nonmotor seizures (1/36, 3%). One-third (12/36, 33%) of these patients required gait aids for ambulation due to prior occurrences of falls. Seizure-related falls resulted in injuries in 18/30 (60%), ranging from skin lacerations (n=9), soft tissue bruises (n=8), subgaleal hematoma (n=1), joint dislocations (n=2), bone fractures (n=5) to life-threatening intracranial hemorrhage (n=1). The injuries occurred most frequently in drop attacks (8/9, 89%) but were also present in facio-brachio-crural dystonic seizures (9/21, 43%) and brachio-crural seizures (1/3, 33%). Seizure-related falls or near falls resolved with immunotherapy (IMT) in 25/36 (69%) whereas the responsiveness to anti-seizure medication (ASM) alone was poor (3/36, 8%). Spontaneous resolution was observed in a minority of patients (4/36, 11%). Among those four patients, three had drop attacks. The median Modified Rankins Scale (mRS) at the peak of the illness was three (range two to five). Upon gait recovery, the median mRS improved to one.
Conclusions:
Our study demonstrates that seizure-related falls and gait disturbance are common in LGI1-IgG autoimmune encephalitis. Early diagnosis, prompt immunotherapy initiation, and proper counseling are key to improving functional outcomes and preventing secondary injuries.
Funding: None.