18975

2014

JPOXXX10.1177/1043454213518975Journal of Pediatric Oncology NursingRoss et al.

Article

Cerebellar Mutism After Posterior Fossa Tumor Resection: Case Discussion and Recommendations for Psychoeducational Intervention

Journal of Pediatric Oncology Nursing 2014, Vol. 31(2) 78­–83 © 2014 by Association of Pediatric Hematology/Oncology Nurses Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1043454213518975 jpo.sagepub.com

Sarah G. Ross, PhD1, Lisa Northman, PhD1, Marybeth Morris, EdM1, Adam L. Green, MD1,2, and Nicole J. Ullrich, MD, PhD1,2

Abstract Cerebellar mutism (also known as posterior fossa syndrome) is a relatively common complication of posterior fossa surgery for primary brain tumors in children. Many children with cerebellar mutism experience long-term adverse neurological, cognitive, and psychological sequelae and require extensive interdisciplinary support. This study illustrates a typical case of cerebellar mutism in a child after resection of medulloblastoma, followed by a review of associated symptoms, clinical course, and modulating factors. Additionally, recommendations for providing educational support to children with cerebellar mutism are explored. Keywords cerebellar mutism syndrome, posterior fossa syndrome, posterior fossa tumor, speech disorder, psychoeducation, neurocognitive function

Introduction Cerebellar mutism (also known as posterior fossa syndrome) is a relatively common complication of posterior fossa surgery for primary brain tumors in children. It is estimated that the overall incidence is 11% to 29% and that patients with medulloblastomas and/or brainstem invasion may be at greater risk (Gudrunardottir, Sehested, Juhler, & Schmiegelow, 2011; Robertson et al., 2006; Turgut, 2008; Wells, Walsh, Khademian, Keating, & Packer, 2008). Timing of onset is typically in the first 24 to 48 hours postoperatively, and characteristic features include diminished speech output or complete loss of speech with dysarthria and linguistic difficulties. Secondary features include emotional lability, apathy, neurobehavioral abnormalities, disturbance in motor functioning, and ongoing neurocognitive impairment (Palmer et al., 2010; Parent & Scott, 2011). There is variability in both severity of symptoms, ranging from mild to severe, as well as timing of recovery, with some symptoms more transient and others unresolving. Many children with cerebellar mutism experience long-term adverse neurological, cognitive, and psychological sequelae. Children diagnosed with cerebellar mutism, therefore, often require extensive interdisciplinary intervention to support recovery and

long-term functioning. Many families and educational teams, however, lack a clear understanding of the diagnosis, clinical features, and recommended services and accommodations required to optimally support the education of children after cerebellar mutism. The goals of this study are to review a typical case of cerebellar mutism in a child after resection of medulloblastoma and to review the timeline of symptoms, clinical course, and modulating factors as well as recommendations for how to support these students in the classroom.

Case Report The patient is a 13-year-old male who presented at age 11 with 1 month of recurrent headaches, vomiting, and ataxia. Neuroimaging studies (Figure 1) demonstrated a midline fourth ventricular mass without evidence of metastatic disease. The patient underwent gross total 1

Pediatric Oncology, Dana-Farber Cancer Institute, Boston, MA, USA Department of Neurology, Boston Children’s Hospital, Boston, MA, USA

2

Corresponding Author: Nicole J. Ullrich, Department of Neurology, Boston Children’s Hospital, 333 Longwood Avenue, Boston, MA 02115, USA. Email: [email protected]

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Figure 1.  Axial FLAIR magnetic resonance imaging through the posterior fossa demonstrates a heterogeneous mass within the midline cerebellum and fourth ventricle (A) with associated enhancement on T1 images after gadolinium administration (B).

resection, with intraoperative imaging showing no evidence of residual tumor. Pathological review revealed medulloblastoma. In the acute postoperative period, the patient spoke several words to his parents and then became mute, with significant mood lability as well as truncal and appendicular ataxia. He was diagnosed with cerebellar mutism at that time. He displayed little verbalization and would say “yes” or “no” only after significant prompting. He was able to utilize a “thumbs up” or “thumbs down” hand gesture to communicate his needs to others but was unable to use a symbol board because of refusal as well as superimposed ataxia that limited the precision of movements. Receptive language appeared intact; however, verbal output consisted mostly of moaning or screeching, which was quite distressing to his immediate family members and led to significant frustration. The patient was unable to sit or stand independently. Speech therapy, communication interventions, and physical and occupational therapies were initiated in the immediate postoperative period, and he was discharged to an acute rehabilitation hospital 1 week later. During his brief 1-week stay in the rehabilitation hospital, supportive therapies were continued, including speech, occupational, and physical therapy. Services were transitioned to the outpatient setting on discharge. He was treated with craniospinal proton beam radiation therapy with a boost to the tumor bed; concurrent vincristine was omitted to allow for neurologic recovery. He was subsequently treated with cyclophosphamide, cisplatin, lomustine, and vincristine, and he completed chemotherapy 14 months after presentation. Late effects of treatment have included bilateral high-frequency hearing loss and growth hormone deficiency; in addition, the patient continues to experience ongoing hemi-motor weakness and appendicular and axial ataxia.

Neuropsychological testing was performed after completion of treatment and demonstrated significant variability in cognitive function. Deficits were noted in the areas of processing speed, speed of verbal and written output, word-finding, reading fluency and comprehension, nonmeaningful auditory memory, and fine and gross motor abilities. Relative strengths were noted in verbal reasoning, naming, and math skills. Additionally, the patient and his parents reported ongoing anxiety about treatment-related late effects. He returned to school on a shortened-day schedule after completion of treatment. A comprehensive Individualized Education Program was developed by the school team, parents, and a hospital-based school liaison program. School-based services included a 1:1 paraprofessional to support safety and mobility, speech therapy, physical therapy, occupational therapy, reading and math special education services, and extended school year programming. In addition, he was provided classroom accommodations that included an FM system, modified/reduced workload, hard copies of lecture notes, text-to-speech assistive technology, and extended time for class work and testing. He continued to receive speech and physical therapy as an outpatient. Ongoing psychological support was provided through a hospital-based provider during medical appointments.

Discussion Since the initial description in 1985 by Rekate and colleagues (Rekate, Grubb, Aram, Hahn, & Ratcheson, 1985), cerebellar mutism syndrome has been recognized as a unique postoperative complication after posterior fossa tumor resection in children. In 2 large multicenter studies that prospectively screened for cerebellar mutism in children with either standard risk medulloblastoma or high-risk medulloblastoma/primitive neuroectodermal tumor, cerebellar mutism occurred in 107/450 children (24%) and was rated as mild in 8%, moderate in 49%, and severe in 43% (Robertson et al., 2006; Turgut, 2008). At 1 year after initial diagnosis, sustained deficits in cognitive, speech, and motor abilities were noted in a significant proportion of patients. Cerebellar mutism typically presents 1 to 5 days after surgery, with diminished speech progressing to the defining symptom of transient mutism, or the complete absence of speech in conscious patients (Parent & Scott, 2011; Wells et al., 2008). Mutism may take several months to resolve and is typically followed by a period of dysarthria. This often results in a characteristic cerebellar speech pattern accompanied by loud, monotonous speech with slowed rate and rhythm (Parent & Scott, 2011). Along with dysarthria, patients may also experience speech apraxia, or the reduced ability to plan and

80 coordinate speech leading to difficulty in using speech consistently and correctly. Some patients also have difficulty swallowing, due to weakened muscles and poor muscle control. Physically, patients often display axial and/or appendicular ataxia, with impaired planning and coordination of motor movements as well as hypotonia (Wells et al., 2008). This results in difficulties with balance and fluent movement and can often present safety risks in navigating areas at school and in the environment that require significant coordination (eg, stairways). Patients with cerebellar mutism often also experience ongoing extreme emotional lability, or frequent changes in mood, which can be quite distressing to patients and their families. This can present as frequent irritability, sadness, anger, and even elation. Finally, there are many, often sustained, neurocognitive deficits after cerebellar mutism, including impulsivity, disinhibition, inattention, difficulty with complex problem-solving, and personality changes (Palmer et al., 2010; Parent & Scott, 2011). There are few studies that have evaluated the anatomical and clinical variables that increase risk for development of cerebellar mutism. It has been proposed that injury to the dentato-thalamo-cortical pathway and disrupted communication between the right cerebellum and left frontal cortex may contribute to speech and language deficits in children with cerebellar mutism (Law et al., 2012; Ozgur, Berberian, Aryan, Meltzer, & Levy, 2006; Pollack, Polinko, Albright, Towbin, & Fitz, 1995). Other factors that may contribute include edema and postoperative swelling, axonal injury due to surgical manipulation, cerebellar/cerebral perfusion deficits, and tumor invasion of the brainstem (Gudrunardottir et al., 2011; Korah et al., 2010; Law et al., 2012). Duration of symptoms in cerebellar mutism ranges from months to years; some deficits may never fully resolve (Korah et al., 2010). In a study examining recovery in patients 1 year after diagnosis of cerebellar mutism, children initially rated in the “severe” range had persistent ataxia (92%), speech and language dysfunction (66%), and cognitive impairment (59%; Robertson et al., 2006). When compared with children who had undergone posterior fossa tumor resection without cerebellar mutism, there is an increased rate of dysarthria, speech dysfluency, and slow rate of speech (Huber, Bradley, Spiegler, & Dennis, 2006). Given the persistence and degree of physical and cognitive impairment associated with cerebellar mutism, comprehensive school-based support is crucial in assisting these patients.

School-Based Recommendations Children with cerebellar mutism present in school settings with a variety of academic, neurocognitive, physical, and

Journal of Pediatric Oncology Nursing 31(2) psychological needs that may also be contributed to by the presence and location of their tumor, tumor resection, and previous treatment modalities (including chemotherapy, cranial irradiation, and/or stem cell transplant). Academic success requires thoughtful and comprehensive planning by the school’s multidisciplinary team, the child’s parents, and often additional input from the child’s medical team. Most children affected by cerebellar mutism (as well as those affected by a primary brain tumor) are eligible for special education services in school settings under the Individuals with Disabilities Education Improvement Act of 2004 (IDEIA) or educational accommodations under Section 504 of the Rehabilitation Act of 1973. IDEIA requires that individualized educational programming be provided for children who have long-term medical conditions that impair their academic and/or social functioning (IDEIA, 2004).

Assessment Assessment in several areas of functioning is required to develop an appropriate educational program for children with cerebellar mutism. Within the school setting, recommended areas of assessment include cognition (IQ testing), academic achievement, socioemotional functioning, gross and fine motor skills, speech and language skills, adaptive skills, orientation and mobility, vision and hearing, and assistive technology. In addition to school-based assessments, these children often require an outside neuropsychological evaluation to further investigate areas of attention, cognitive and executive functioning, memory, processing, psychological status, and other neurocognitive areas. Because the prolonged course of recovery often includes inpatient stays in rehabilitation hospitals, these children may have also had evaluations from other fields before their return to school (eg, speech and language, physical therapy). These evaluations should be incorporated as part of the data used to develop an effective plan for the child.

Individualized Educational Program Children who qualify for special education services will likely fall under the categories of Neurological, Traumatic Brain Injury, or Other Health Impaired (OHI), depending on the special education terminology used in the state in which the child is enrolled in school. The multidisciplinary team should then develop an Individualized Education Program (IEP) specific to the recommendations from the evaluations completed by the team members. Generally, children with cerebellar mutism require direct services and accommodations in the areas of speech and language, gross and fine motor skills, executive functioning, and social-emotional needs. Table 1 includes

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Ross et al. Table 1.  School-Based Recommendations for the Treatment of Common PFS-Related Physical, Cognitive, and Psychological Difficulties. Common Difficulties

Interventions

Speech and language  Apraxia  Dysarthria   Expressive language   Receptive language       Gross and fine motor  Ataxia  Dysphagia   Muscle weakness

  Speech therapy to:   Increase fluency of speech practice phrases used frequently   Develop nonverbal ways of communicating   Develop age-appropriate vocabulary   Develop breath control Break complex directions into smaller parts Provide written directions in addition to verbal   Physical therapy to improve gross motor ability and tone Occupational therapy to improve fine motor skills, including daily adaptive skills Speech therapy to address texture and motor issues that are affecting swallowing Adaptive physical education Break complex motor tasks into smaller steps Use of assistive motor devices 1:1 paraprofessional to ensure mobility safety at school Use of an elevator Use of assistive technology Transition between classes or areas of the building during less busy time periods Additional time for transitions   Special education instruction in areas of deficit Frequent breaks and extra time for task completion Extra time to process information and to provide verbal responses Modified curriculum Relate new information to previously learned concepts Provide new information in small chunks Frequent repetition Break common academic tasks into steps and provide steps in? a visual format Explicit instruction in organizational strategies for learning and daily life Small group testing   Cognitive-behavioral therapy to increase behavioral regulation skills Social skill groups or “lunch bunches” to build skills necessary to make and maintain friendships School-based leisure/hobby activities to promote self-esteem and leadership

  Visual-motor integration               Cognitive and executive functioning   Processing speed   Sustaining, shifting, and dividing attention  Organization  Problem-solving   Task initiation and completion           Social-emotional   Mood swings   Social skills   Sensory-integration issues Note. PFS = posterior fossa syndrome.

common difficulties for patients with cerebellar mutism in these 4 areas and recommendations for school-based interventions and accommodations to meet the needs of these children. In addition to the 4 areas listed, many of these children will need direct intervention in academic areas, as patients treated for brain tumors are at a greater risk for academic difficulties (Bruce, Newcombe, & Chapman, 2012; Daly, Kral, & Brown, 2008; Mabbott et al., 2005; Mulhern et al., 2005). A study that examined academic progress in children who received radiation therapy for posterior

fossa tumors indicated that children’s academic performance declined in the areas of spelling, mathematics, and reading (Mabbott, et al., 2005). In addition to academic deficits related to diagnosis and treatment, these students will have likely missed significant portions of academic instruction due to hospitalizations and recovery periods. If a child is attending school on a part-time basis, homebound instruction may be appropriate to support academic progress in addition to the direct academic support that he or she is receiving at school (Shaw & McCabe, 2008).

82 In addition to yearly multidisciplinary team meetings to discuss progress in all targeted areas, children with cerebellar mutism will need frequent progress monitoring and reassessment throughout the school year to evaluate gains and/or losses in skill, and to modify the IEP accordingly.

Additional Considerations Children diagnosed with cerebellar mutism (and brain tumors in general) face many challenges as they recover from treatment, adjust to physical and emotional losses, and return to the school setting. As discussed, these children often present with neurocognitive, physical, and social deficits that make navigating the school setting considerably difficult. Children with brain tumors are more likely to experience academic and social difficulties (including isolation and bullying) than peers who have not experienced a chronic illness; these children are at greater risk to experience symptoms of anxiety and depression (Daly et al., 2008; Mabbott et al., 2005; Turner, Rey-Casserly, Liptak, & Chordas, 2009; Upton & Eiser, 2006). Children with brain tumors report that keeping up with schoolwork, adjusting to changes in physical abilities and appearance, and building and maintaining social relationships are main challenges during and after treatment (Freeman, O’Dell, & Meola, 2003). Parents discuss many concerns and challenges as they support their child’s return to school after treatment, including ensuring physical safety, making certain that school personnel have appropriate knowledge and awareness about diagnosis and effects of treatment, obtaining appropriate academic supports, and finding appropriate equipment and transportation (Freeman et al., 2003). In addition to multidisciplinary team meetings and educational plan development discussed above, additional recommendations to meet the needs of these children include (a) open and consistent communication between the school team, medical team, and parents; (b) advanced planning and preparation, especially during critical transition years (eg, elementary to middle school); (c) promotion of social opportunities within the school setting; and (d) ongoing school-based education and awareness activities (Bruce, Chapman, MacDonald, & Newcombe, 2008; Bruce et al., 2012). Children with cerebellar mutism present with many challenges, and ongoing, intensive interdisciplinary care is critical for their success. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Journal of Pediatric Oncology Nursing 31(2) Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.

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Author Biographies Sarah G. Ross, PhD, is completing her postdoctoral fellowship in pediatric psychology under the supervision of Dr Lisa Northman. She has academic and clinical training in school psychology and currently provides school consultation to children affected by central nervous system disease and/or treatment. Lisa Northman, PhD, is a clinical psychologist with expertise in neuropsychology and neurocognitive late effects of oncology treatment. She has provided school consultation to children and their families at Dana-Farber for the past 7 years. Marybeth Morris, EdM, is a school psychologist with expertise in assessment, neuropsychology, and neurocognitive late effects of treatment. She has 6 years of experience working with children affected by cancer and 5 years of experience providing services in the school setting. Adam L. Green, MD, is a pediatric hematologist-oncologist at Boston Children’s/Dana-Farber Cancer and Blood Disorders Center who specializes in neuro-oncology. Nicole J. Ullrich, MD, PhD, is a child neurologist who specializes in neurologic complications of cancer in children and neurologic outcomes of children with primary brain tumors.

Cerebellar mutism after posterior fossa tumor resection: case discussion and recommendations for psychoeducational intervention.

Cerebellar mutism (also known as posterior fossa syndrome) is a relatively common complication of posterior fossa surgery for primary brain tumors in ...
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