TREATMENT
Little is still known about how to treat the condition, and randomized controlled trials are lacking.
Medication
Anectotal reports have been published about the effect of various medication on individual patients. The dopamine agonist bromocriptine [1, 2] and the calcium antagonist nimodipine [3] have been suggested as medical treatments of mutism and prevention of ischemia due to vasospasms, while the anitconvulsants carbamazepine and oxcarbazepine have been recommended as mood stabilizers [4] and melatonin for neuroprotection [5]. The administration of the gamma-aminobutyric acid-A agonist zolpidem coincided with prompt clinical improvements in one post-operative pediatric CMS patient, which parallels improvements seen with use of zolpidem in other neurologic and psychiatric disorders such as akinetic mutism, catatonia, minimally conscious states and coma [6]. The administration of the selective serotonin reuptake inhibitor fluoxetine coincided with clinical improvements in another patient, but the drug that has previously been used to treat elective mutism [7]. The improvements described in these two cases may have been due to spontaneous resolution of symptoms rather than a drug effect, so further investigation is needed. Many brain tumor patients routinely receive corticosteroids, but the role that they may play in the prevention and/or treatment of PFS is uncertain. Peri-operative steroids have been recommended as they may lessen edema around the resection cavity [8] (see also under Prevention), and pulsed methylprednisolon has been reported to precede the resolution of symptoms of POP-CMS in several patients with rotavirus-associated encephalitis/cerebellitis [9, 10]. However, information about doses, timing and effects of peri-operative steroids on the course of POP-CMS in cerebellar tumor patients is not found anywhere in the literature, which underscores the need for systematic assessment.
Other treatments
Physical, occupational and speech therapy are considered important [4, 11], as is the development of specific nursing strategies for post-operative pediatric CMS patients and their families. These do not only involve managing acute symptoms of the patient and supporting the rest of the family through a period of intense stress, but also coordinating efforts between doctors, speech therapists, multidiciplinary rehabilitation teams and psychologists, as well as providing information to educational services and community representatives [11]. Neuroscience nurses may play an instrumental role in this process, and ultimately enable optimal patient and family outcomes [11, 12]. Highly individualized approach to treatment planning is warranted [13], but documentation of the effects of such therapies should also be studied and reported.
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Definitions, Incidence, Symptoms, Anatomy, Pathophysiology, Imaging findings, Risk factors, Prevention
1. Caner H, Altinors N, Benli S, Calisaneller T, Albayrak A (1999) Akinetic mutism after fourth ventricle choroid plexus papilloma: treatment with a dopamine agonist. Surg Neurol 51: 181-184
2. Catsman-Berrevoets CE, van Dongen HR, Zwetsloot CP (1992) Transient loss of speech followed by dysarthria after removal of posterior fossa tumour. Dev Med Child Neurol 34: 1102-1109
3. Turgut M (1998) Transient "cerebellar" mutism. Childs Nerv Syst 14: 161-166
4. Beckwitt TS, Krieger MD, O'Neil S, Jubran R, Tavare CJ (2012) Symptoms before and after posterior fossa surgery in pediatric patients. Pediatr Neurosurg 48: 21-25
5. Turgut M (2010) Cerebellar mutism. J Neurosurg Pediatr 6: 303-304
6. Shyu C, Burke K, Souweidane MM, Dunkel IJ, Gilheeney SW, Gershon T, Khakoo Y (2011) Novel use of zolpidem in cerebellar mutism syndrome. J Pediatr Hematol Oncol 33: 148-149
7. Akhaddar A, Salami M, El Asri AC, Boucetta M (2012) Treatment of postoperative cerebellar mutism with fluoxetine. Childs Nerv Syst 28: 507-508
8. Souweidane MM (2010) Posterior fossa syndrome. J Neurosurg Pediatr 5: 325-326
9. Kobayashi S, Negishi Y, Ando N, Ito T, Nakano M, Togari H, Wakuda M, Taniguchi K (2010) Two patients with acute rotavirus encephalitis associated with cerebellar signs and symptoms. Eur J Pediatr 169: 1287-12
10. Kubota T, Suzuki T, Kitase Y, Kidokoro H, Miyajima Y, Ogawa A, Natsume J, Okumura A (2011) Chronological diffusion-weighted imaging changes and mutism in the course of rotavirus-associated acute cerebellitis/cerebellopathy concurrent with encephalitis/encephalopathy. Brain Dev 33: 21-27
11. Mortimer DS (2011) Clinical case study: a 4-year-old boy with posterior fossa syndrome after resection of a medulloblastoma. J Neurosci Nurs 43: 225-229
12. Parent E, Scott L (2011) Pediatric posterior fossa syndrome (PFS): nursing strategies in the post-operative period. Can J Neurosci Nurs 33: 24-31
13. Morgan AT, Liegeois F, Liederkerke C, Vogel AP, Hayward R, Harkness W, Chong K, Vargha-Khadem F (2011) Role of cerebellum in fine speech control in childhood: persistent dysarthria after surgical treatment for posterior fossa tumour. Brain Lang 117: 69-76