Balint’s Syndrome in Setting of Idiopathic Bilateral Occipital Lobe Periodic Discharges
Abstract number :
1.416
Submission category :
18. Case Studies
Year :
2023
Submission ID :
95
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Usaamah Khan, MD – Massachusetts General Hospital, Brigham and Women's Hospital, Harvard Medical School
Saef Izzy, MD – Faculty, Neurocritical Care, Neurology, Brigham and Women's Hospital, Harvard Medical School
Rationale: Balint’s Syndrome is a rare condition that presents with a triad of optic ataxia, oculomotor apraxia, and simultagnosia. The underlying pathology can be a broad range of ischemic, autoimmune, infectious, or even neurodegenerative etiologies. We present a case of a young female who presented with new onset generalized convulsive seizure and a clinical course complicated by persistent symptoms of Balint’s Syndrome in the setting of refractory bilateral occipital lobe discharges.
Methods: A 52-year-old female with alcohol use disorder presented with first-time seizure in the setting of presumed alcohol withdrawal. She was treated with Levetiracetam with no further seizures however developed fluctuating mental status, optic ataxia, oculomotor apraxia, and simultanagnosia. Magnetic Resonance Imaging (MRI) of the brain revealed diffusion restriction of bilateral parietal and occipital lobes as well as T2 hyperintensity of mamillary bodies and peri-aqueductal gray area with consideration for Wernicke’s Encephalopathy (WE) and Posterior Reversible Encephalopathy Syndrome (PRES) however clinical and radiographic features not consistent with either etiology. Electroencephalography (EEG) revealed occipital bilateral independent discharges (BIPDs) in the 1 to 1.5Hz range and treated with escalating anti-seizure medications (ASM) including Ketamine infusion, Levetiracetam, Lacosamide, Clobazam and Perampanel with improvement in EEG and symptoms.
Results: Extensive Blood and Cerebrospinal fluid (CSF) infectious and inflammatory workup, auto-immune encephalitis panel, malignancy, and paraneoplastic workup were all negative. A whole-body Positive Emission Tomography (PET) study revealed focal FDG uptake in the right occipital cortex and Magnetic Resonance Spectroscopy (MR Spect) revealed nonspecific decreased N-Acetylaspartate and increased lactate levels within signal abnormality. The patient was treated with empiric steroids and Intravenous Immunoglobulin (IVIG) without marked improvement. Ultimately, with continued ASM there was an improvement in MRI changes and symptoms. The patient was discharged to rehabilitation with outpatient follow-up.
Conclusions: Balint’s Syndrome in the setting of idiopathic occipital lobe periodic discharges is a rare occurrence. The radiographic occipital lobe signal changes raised concern for a possible underlying inflammatory/encephalitic process driving the high epileptiform discharge burden versus the possibility of ongoing radiographic findings due to cerebral metabolic changes secondary to ictal activity. In such a scenario, extensive workup to identify reversible etiologies and aggressive treatment is warranted given disabling symptoms in an otherwise healthy patient. Though a cause was not identified, the patient's classic triad of symptoms and high clinical index for suspicion led to a comprehensive workup and aggressive treatment allowing for improvement in the patient’s condition. The case demonstrates the complex investigations and diagnostic considerations in adult new onset occipital lobe epilepsy.
Funding: No funding.
Case Studies