Posterior Fossa Syndrome after a Vermian Stroke: A New Case and Review of the Literature

Abstract

The posterior fossa syndrome (PFS) is a well-known clinical consequence of posterior fossa surgery that has only been reported in a limited number of cases with a nontumoral etiology. It consists of transient cerebellar mutism, behavioral abnormalities and personality changes. We describe a 12-year-old child who developed transient cerebellar mutism associated with behavioral and emotional symptoms following rupture of a vermis arteriovenous malformation (AVM). Following the stroke, the girl experienced a 24-hour symptom-free interval. After that, she became mute and her emotional state was characterized by severe anxiety, irritability and withdrawal. After 3 days, mutism resolved and dysarthria became apparent. Two weeks after stroke, the AVM was surgically removed and the postoperative course was uneventful. This case is the first reported in which the PFS occurred after focal nonsurgically induced cerebellar damage.

Complete Ocular Paresis in a Child with Posterior Fossa Syndrome

“…Since then more than 200 cases have been reported in the literature. The majority of cases are in patients undergoing resection of medulloblastoma; however, patients with traumatic vascular and infectious etiologies have also been described [2]. PFS has been reported to occur in about 15–20% of patients undergoing posterior fossa surgery for tumor removal [3,4].…”

“…Patients who have had surgical interventions for tumors that infiltrate the vermis, brainstem, deep cerebellar nuclei, cerebellar outflow tracts or some combination of these structures seem to be at the greatest risk of developing PFS [5]. The most common symptoms of the syndrome consist of emotional lability, transient mutism, behavioral changes, and ataxia [2]. Not all aspects of PFS in childhood are transient, and it is now recognized that some degree of neuropsychological dysfunction may be long-lasting [6].…”

Identifying quantitative imaging features of posterior fossa syndrome in longitudinal MRI

“…1 This syndrome, also known as cerebellar mutism syndrome, describes a set of neurological symptoms that may develop from 24 to 107 h after surgery. 2,3 Children suffering from PFS characteristically suffer from disturbance in speech and mutism but may also suffer from loss of muscle tone, incontinence, strabismus (crosseyed), dysphagia, and personality changes such as anger, apathy, melancholy, crying, and screaming. 2 The development of such a syndrome in children hinders their development and highly impacts their quality of life.…”

Tractografía de la vía dento-rubro-talámica en mutismo cerebeloso postoperatorio en niños con tumores de fosa posterior

“…El primero en describirlo fue Rekate en 1985 2 ; principalmente como un déficit postoperatorio en cirugías de fosa posterior 3,4 , aunque también se ha descrito secundario a infecciones [5][6][7][8] , trauma 9,10 o eventos isquémicos [11][12][13] . Es una complicación relativamente común después de una cirugía de fosa posterior (se ha descrito una incidencia entre el 11 y el 29% de los casos) 4,14,15 aunque su verdadera incidencia es difícil de determinar debido a problemas en la terminología y una definición variable del síndrome 16 .…”