A Case of Bilateral Spiller Syndrome

Upon awakening from sleep, a 56-year-old man with diabetes and hypertension, who had never experienced neurological issues before, presented with a sudden weakness in his right upper and lower limbs. This weakness later progressed to include the left side as well. Upon admission, his pulse was at 76 beats per minute, and his blood pressure measured 146/90 mmHg. He became drowsy and required intubation. His pupils were normal, with bilateral nystagmus and mild up gaze restriction were observed, along with right upper motor neuron facial palsy and bilateral tongue weakness. Motor examination revealed quadriplegia (0/5 in all limbs) along with bilateral extensors planters. Sensory examination displayed bilateral impaired proprioception. Tests for cerebellar function, coordination, and balance were impossible to perform. His National Institutes of Health Stroke Scale (NIHSS) score was determined to be 16 out of 42. The initial metabolic measurements revealed a serum glucose level of 440 mg/dl with negative e urinary ketone bodies, and a serum sodium level of 135 mg/dl. The white blood count was 11400, with 76% neutrophils and a normal platelet count. Liver, kidney, and thyroid functions were within normal limits. A MRI of the brain conducted upon admission showed no abnormalities, although an angiogram indicated non visualization of the left ACA’s A1 segment, attenuated left PCA, and non-visualization of the right vertebral artery [Figure 1]. The patient was tentatively diagnosed with MRI-negative acute ischemic stroke. Due to the stroke occurring while the patient was asleep, the lack of MRI findings, and the time elapsed beyond the 4.5-hour window for thrombolysis, treatment was initiated with a loading dose of antiplatelets and atorvastatin, followed by a maintenance dose.Figure 1: MRI brain axial section showed diffusion restriction in bilateral medial medulla (a) with ADC reversal (b) as marked with white arrow and T2* showed hyperintensity in bilateral medial medulla (c).MR angiogram showed non-visualization of the right vertebral artery (d)The MRI brain was repeated revealed diffusion restriction in the bilateral medial medullary region, causing ADC reversal and giving the appearance of “heart-shaped” hyperintensity in the bilateral medial medulla with T2 flair hyperintensity. A diagnosis of bilateral medial medullary infarct (BMMI) syndrome was considered. The patient had an early tracheostomy and received treatment to control his sugar levels, as well as standard therapy for his stroke. After 10 days, he was transferred to a rehabilitation center for continued care, as he remained quadriplegic and needed oxygen supplementation through a T piece. Consent has been taken from the relatives. Discussion Medial medullary infarcts are a rare type of stroke, usually occurring unilaterally, with bilateral cases being extremely uncommon. This stroke presentation accounts for less than 0.5% of all cerebral infarcts.[1] Medial medullary/dejerine syndrome causes contralateral hemiplegia/paresis due to pyramidal tract invovlement and ipsilateral tongue weakness due to 12th nerve involvement where as Spillers syndrome affects ipsilateral 12th nerve nucleus and its fibres causing tongue weakness, contralateral corticospial tract causes contralateral hemiplegia/paresis, and medial lemniscus (hemi-medial medulla involvement) contralateral loss vibration and proprioception.[2] Atherosclerotic disease of the vertebral and anterior spinal artery or its branches is the most common cause, and it is associated with spiller’s syndrome. Spiller syndrome, an uncommon variant of medial medullary infarction. Here we demonstrate a patient with bilateral spiller syndrome. Unfortunately, it has a generally poor prognosis. The classic “heart appearance” in the medulla on diffusion-weighted MRI is a characteristic MRI brain finding.[3] Other names given to the “heart appearance” are “Airpod sign”[4] or “Letter Y sign”.[5] In our case, Guillain-Barre syndrome (GBS) and brainstem encephalitis are other clinical differential diagnoses that may resemble spillers syndrome, but they typically develop sub acutely, and CSF analysis can be helpful in confirming the diagnosis. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.