Bilateral crossed cerebello-cerebral diaschisis and mutism after surgery for cerebellar medulloblastoma

Abstract

A 7-year-old boy developed mutism after surgery for cerebellar medulloblastoma. Postoperative magnetic resonance imaging (MRI) showed atrophy of the cerebellar vermis and both cerebellar hemispheres, predominantly on the right side. Single photon emission computed tomography (SPECT) with technetium-99m-ethyl cysteinate dimer (Tc-99m ECD) revealed decreased cerebral blood flow (CBF) in the bilateral thalami, bilateral medial frontal lobes, and left temporal lobe in addition to the cerebellar vermis and both cerebellar hemispheres when mutism was manifest, indicating the existence of bilateral crossed cerebello-cerebral diaschisis (BCCCD). Circulatory disturbance in both cerebellar hemispheres secondary to tumor resection probably caused BCCCD in both cerebral hemispheres, predominantly in the left, via the dentatothalamocortical pathway (DTCP). With recovery of his mutism, CBF increased in the right thalamus, bilateral medial frontal lobes and left temporal lobe. Thus BCCCD was improved, with only a slight decrease in CBF still persisting in the left thalamus. The mechanism of mutism may have involved damage to the cerebellar vermis (the site of incision at operation), the left dentate nucleus (heavily infiltrated by the tumor) and the right dentate nucleus of the cerebellum (affected by circulatory disturbance secondary to acute postoperative edema). The SPECT findings suggested that mutism was associated with BCCCD-induced cerebral circulatory and metabolic hypofunction in the supplementary motor area mediated via the DTCP.

Cerebellocerebral Diaschisis and Postsurgical Posterior Fossa Syndrome in Pediatric Patients

“…We fully agree that the study of Miller et al 1 adds important evidence to current insights in the possible cause and pathophysiologic mechanism underlying the PFS, but we would like to point out that CCD has already been reported on several previous occasions as a possible explanation for transient postoperative mutism 2,3 and for the constellation of neurobehavioral and affective symptoms characterizing PFS in the pediatric population. [4][5][6] For instance, Mariën et al (2001Mariën et al ( , 2003 4,6 reported the preliminary results of a prospective study in which the pathophysiologic role of CCD in PFS was explored.…”

Cerebellar mutism

“…63 Multiple studies have supported the theory of cerebellocerebral diaschisis using different imaging techniques that revealed decreased blood flow, metabolic action, and function within supratentorial structures that play a crucial role in speech production, such as the thalami, inferior frontal gyrus, and temporal lobe. 26,34,63,65,83 In almost every case the abnormal findings returned to normal when mutism resolved.…”

“…Later 26 Erşahin and colleagues reported on 1 patient with right cerebellar hemisphere hypoperfusion. Sagiuchi et al 83 reported a case of CM in a 4-year-old boy with atrophy of both cerebellar hemispheres, predominately the right hemisphere, on MRI and circulatory disturbance in both hemispheres secondary to tumor resection in SPECT scans. Besides SPECT perfusion imaging, an alternative technique called arterial spin-labeling perfusion MRI was used on a girl with postoperative CM and showed bilateral hypoperfusion in the cerebellar hemisphere, thalamus, and frontal lobe.…”

“…93 Riva and Giorgi 81 supported that vermian lesions led to 2 different profiles: postsurgical CM, which evolved into speech disorders or language disturbances similar to agrammatism, and behavioral disturbances (ranging from irritability to autistic behavior) supporting the role of vermis as part of a "cerebellar limbic system." 94 Sagiuchi et al 83 reported a case of CM that was associated with atrophy of the vermis on MRI and decreased blood flow in the same region on the SPECT scan, and proposed that CM resulted from damage to the vermis with or without a bilateral cerebellar hemisphere lesion. In addition, Frassanito et al 31 reported a case of CM after spontaneous intratumoral bleeding involving the upper cerebellar vermis and believed that the localization of bleeding provided confirmation of the role played by the upper vermis in speech control.…”

“…87,109 Facial and abducent nerve palsies have been reported but as coexisting findings. 83,87 Patients with urinary incontinence and loss of bowel control have also been reported. 77 Von Hoff et al 106 described the presence of ataxia, nystagmus, and dysmetria in patients after posterior fossa surgery as the "cerebellar syndrome.…”