Atypical Posterior Reversible Encephalopathy Syndrome with Obstructive Hydrocephalus and Impending Tonsillar Herniation.
Juan Solano1, Saman Zafar2, George Newman3
1Neurology, Einstein Healhcare Network, 2Einstein Medical Center Philadelphia, 3Einstein Medical Center
Objective:
OBJECTIVE AND IMPORTANCE: Rhombencephalic edema with hydrocephalus has rarely been described as a manifestation of hypertensive emergency. We present an unusual case of brain edema with hydrocephalus secondary to hypertensive emergency.
Background:
CASE DESCRIPTION: A 33-year-old male with uncontrolled hypertension and CKD Stage IV presented to hospital with occipital headache and vomiting. His BP was 230/160. He was treated with Nicardipine infusion. CT head showed cerebellar vasogenic edema and mild tonsillar herniation with partial effacement of the fourth ventricle. MRI confirmed these findings, there was disproportionate dilatation of the lateral and third ventricles with periventricular T2 hyperintense signal consistent with transependymal CSF flow. The headache and neck pain were persistent. The patient was upgraded to Neurocritical Care Unit for monitoring and continued IV BP treatment. Given concerns for incipient tonsillar herniation, Mannitol and Hemodialysis [HD] were added to his medical treatment. After these interventions, his headache and blood pressure management improved. Two days later, CT showed significant interval improvement of hydrocephalus, cerebral edema and posterior fossa crowding.
Results:
DISCUSSION: This was an unusual case of extension of PRES to the posterior fossa, leading to hydrocephalus and a vicious cycle of worsening rise in intracranial pressure, leading to worsening refractory systemic hypertension, end organ damage, need for hemodialysis, impending tonsillar herniation and risk of death.
Conclusions:
CONCLUSION: This presentation and improvement were compatible with the final diagnosis of an atypical presentation of Posterior Reversible Encephalopathy Syndrome. In this case, reduction of blood pressure, hyperosmolar therapy and dialysis resulted in improvement of the symptoms as well as radiological resolution of hydrocephalus. Aggressive medical treatment was the best approach in this case, but neurosurgical intervention would have been necessary if the patient had not responded to these measures.