Archives of Medicine and Health Sciences

: 2017  |  Volume : 5  |  Issue : 1  |  Page : 131--132

The heart of the matter: Acute quadriplegia with respiratory paralysis - bilateral medial medullary infarction

Bhaskara P Shelley 
 Department of Neurology, Yenepoya Medical College, Yenepoya University, Mangalore, Karnataka, India

Correspondence Address:
Bhaskara P Shelley
Department of Neurology, Yenepoya Medical College, Yenepoya University, Mangalore - 575 018, Karnataka


The clinicoradiologic correlate of bilateral medial medullary infarction is described. This is a rare clinical entity of vertebrobasilar stroke syndrome with catastrophic consequences and a poor functional prognosis. Since the initial symptom is quadriplegia, the clinical diagnosis without neuroimaging can be challenging with a potential for misdiagnosis as Guillain–Barré syndrome or brainstem encephalitis in the early stages. The teaching neuroimage of the “heart appearance” sign is revisited.

How to cite this article:
Shelley BP. The heart of the matter: Acute quadriplegia with respiratory paralysis - bilateral medial medullary infarction.Arch Med Health Sci 2017;5:131-132

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Shelley BP. The heart of the matter: Acute quadriplegia with respiratory paralysis - bilateral medial medullary infarction. Arch Med Health Sci [serial online] 2017 [cited 2022 Sep 30 ];5:131-132
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The patient was a 70-year-old right-handed woman without any traditional vascular risk factors or medical comorbidities who presented with acute onset left-sided weakness of the body, vomiting, vertigo, difficulty in swallowing, and slurring of speech in the morning about 1 hour on awakening from sleep. Examination was remarkable in that she had an elevated blood pressure of 220/110 mm Hg with Grade 1 hypertensive retinopathy, was alert and conscious with a Glasgow Coma Scale 15/15, Grade 0/5 power in the left upper limb and left lower limb, with left gaze preference. No facial palsy was noted, and extraocular movement was full. The soft palate did not show any asymmetry, but pharyngeal reflex was absent. Tongue protrusion was possible and not deviated. The right lower limb power was 4/5 at the hip, and knee and 1/5 at the ankle with generalized hypotonia, absent deep tendon reflexes, similar to an acute spinal cord shock state, and bilateral extensor plantar reflexes. Sensory system could not be evaluated. After 12 hours of hospital admission, there was worsening weakness on the right side of her body, progressing to Grade 0/5 quadriplegia, developed respiratory distress, desaturation necessitating neurointensive care, emergent intubation, and ventilation. She was still able to communicate with eye movements and blinking. Her hematological and biochemical investigations, electrocardiogram, and transthoracic echocardiogram including cerebrospinal fluid analysis were unremarkable. Suspecting a brainstem stroke, her brain Magnetic Resonance Imaging (MRI) showed a sharply demarcated “heart-shaped” area of hyperintense signal in bilateral anteromedial medullae on axial T2-weighted image [Figure 1] and [Figure 2] with restricted diffusion on diffusion-weighted imaging consistent with acute bilateral rostral medial medullary infarct. Her magnetic resonance angiography did not reveal any vascular abnormalities in the vertebrobasilar system. She was started on Ramipril, dual antiplatelet therapy, Atorvastatin, and parenteral Cerebrolysin. A tracheostomy was done after 2 weeks, remained fully conscious and alert, with Grade 0/5 quadriparesis. She was weaned off the ventilator by the 3rd week of stay in neurointensive care, with improvement of power to 2/5 in both upper and lower extremities. After 4 weeks of admission, she was discharged at the request of family members for continuation of care at a nearby regional hospital.{Figure 1}{Figure 2}

Davison first described a case of bilateral medial medullary infarction (MMI) in 1937, and the disease remained a rare cerebrovascular disorder since after.[1] The rapid confirmation of the diagnosis in patients with “forme fruste” manifestations can be challenging. Early diagnosis is crucial given that the treatment strategies are completely different. Diagnosis of bilateral MMI has now become possible based on brain MRI findings but does prove clinically challenging to diagnose. Bilateral MMI is an extremely rare cerebrovascular accident with morbidity and mortality and a poor prognosis for functional recovery, especially if the infarction is caused by anterior spinal artery (ASA) occlusion. Occlusion of the vertebral or ASA or their branches to the paramedian region of the medulla oblongata is the usual cause of bilateral MMI.[2] The infracted area usually included the pyramidal tracts, medial lemnisci, medial longitudinal fasciculus, hypoglossal nucleus or hypoglossal nerve fibers, and medullary reticular formation bilaterally. The “heart appearance” sign is considered to appear when the infarct occurs in the anterior-medial territory and anterior-lateral territory of the medulla oblongata.

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1Davison C. Syndrome of the anterior spinal artery of the medulla oblongata. Arch Neurol Psychiatry 1937;37:91-107.
2Katoh M, Kawamoto T. Bilateral medial medullary infarction. J Clin Neurosci 2000;7:543-5.