Cerebellar Tumor Surgery Research Articles

THE EFFECT OF PHYSICAL THERAPY INTERVENTION AFTER CEREBELLAR RESECTION

A variety of diseases, including gait ataxia, lack of coordination, diminished dexterity, and unsteady posture, can be brought on by cerebellar dysfunction. This case study looks into how a physical therapy program affected a 40-year-old patient's dynamic balance after cerebellar tumour surgery. The patient complained of losing his balance and having uncoordinated motions on the left side of the body. The Timed Up and Go (TUG) test was used to gauge how the Frenkel coordination exercises and balance training affected dynamic balance following cerebellar tumour removal. The patient's muscle strength in the left-side hip extensors, abductors and adductors, knee flexors and extensors, as well as the left-side shoulder, elbow, and wrist flexion and extension, significantly improved.

Characterising the Long-Term Language Impairments of Children Following Cerebellar Tumour Surgery by Extracting Psycholinguistic Properties from Spontaneous Language.

Following cerebellar tumour surgery, children may suffer impairments of spontaneous language. Yet, the language processing deficits underlying these impairments are poorly understood. This study is the first to try to identify these deficits for four levels of language processing in cerebellar tumour survivors. The spontaneous language of twelve patients who underwent cerebellar tumour surgery (age range 3-24years) was compared against his or her controls using individual case statistics. A distinction was made between patients who experienced postoperative cerebellar mutism syndrome (pCMS) and those who did not. Time since surgery ranged between 11months and 12;3years. In order to identify the impaired language processing levels at each processing level (i.e., lexical, semantic, phonological and/or morphosyntactic) nouns and verbs produced in the spontaneous language samples were rated for psycholinguistic variables (e.g., concreteness). Standard spontaneous language measures (e.g., type-token ratio) were calculated as well. First, inter-individual heterogeneity was observed in the spontaneous language outcomes in both groups. Nine out of twelve patients showed language processing deficits three of whom were diagnosed with pCMS. Results implied impairments across all levels of language processing. In the pCMS-group, the impairments observed were predominantly morphosyntactic and semantic, but the variability in nature of the spontaneous language impairments was larger in the non-pCMS-group. Patients treated with cerebellar tumour surgery may show long-term spontaneous language impairments irrespective of a previous pCMS diagnosis. Individualised and comprehensive postoperative language assessments seem necessary, given the inter-individual heterogeneity in the language outcomes.

Involvement of the cerebellum in the regulation of executive functions in children-Preliminary analysis based on a neuropsychological study of children after cerebellar tumour surgery.

AimPreliminary assessment of executive functions in children with cerebellar lesions, description of their emotional-social functioning and selection of sensitive neuropsychological tools to detect the cerebellar cognitive affective syndrome (CCAS).Materials and methodsThe study group consisted of 10 children after cerebellar tumour surgery. The control group consisted of 10 healthy children, matched for age and sex: The IDS-2 executive functions battery, the Conners 3 ADHD questionnaire, the Autism Spectrum Rating Scales (ASRS) and the International Cooperative Ataxia Rating Scale (ICARS) were used.ResultsStatistical analysis showed statistically significant differences between the experimental and control groups in terms of two dimensions of executive functioning. Children from experimental group was characterised by worse planning and divided attention than healthy controls. Moreover children with cerebellar lesions were characterised by significantly higher levels of some behaviours similar to that observed in autism spectrum disorders, namely difficulties in social relationships, self-regulation of emotions, attention, and greater behavioural rigidity. Test power analysis and estimation of the effect size by the Cohen’s d coefficient indicated that with a slight increase in the size of the experimental group, the probability of detecting statistically significant difference in the executive functions total measure score as well as in several ASRS subscales increased, but not in Conners 3 subscales.ConclusionsCerebellar damage may pose a risk for dysexecutive syndrome and social-emotional problems in children. The IDS-2 executive functions battery and the ASRS test are sufficiently sensitive tools to assess elements of the CCAS in children.

IMG-03. Impact of childhood cerebellar tumor surgery on cognition: Can fMRI serve as a surrogate marker?

BACKGROUND: Childhood cerebellar pilocytic astrocytomas harbour excellent overall survival rates after surgical resection, but the patients may exhibit specific cognitive and behavioural problems emphasising our current understanding of the cerebellar role in cognition. Functional MRI has catalysed the mechanistic insights into brain functional systems and has already been linked with the neuropsychological performance. We aimed to exploit the question whether functional MRI can be used as a surrogate measure for the cognitive outcome assessment of these patients. METHODS: We investigated 13 patients (median age 22.0 years, range 15.2 – 31.7) after a median interval between surgery and examination of 15.0 years (range 4.2 – 20.8) and 16 matched controls. All subjects underwent a functional 3T MRI scans in a resting state condition and a battery neuropsychological tests. RESULTS: Patients showed a significantly increased functional connectivity in the precuneus compared to controls (p<0.05) and at the same time impairments in various domains of neuropsychological functioning such as a lower mean WIE IQ percentile (M = 48.62, SD = 29.14), lower scores in the TMT letter sequencing (M = 49.54, SD = 30.66), worse performance on the WIE subtest Digit Symbol Coding (M = 38.92, SD = 35.29), subtest Symbol Search (M = 40.75, SD = 35,28), and TAP divided attention task (M = 783.92, SD = 73.20). CONCLUSION: Functional MRI has the potential to serve as a surrogate measure for the cognitive outcome assessment. The precuneus brain region is heavily involved in the cerebral associative oversight of cerebellar cognitive function.

Impact of childhood cerebellar tumor surgery on cognition revealed by precuneus hyperconnectivity.

BackgroundChildhood cerebellar pilocytic astrocytomas harbor excellent overall survival rates after surgical resection, but the patients may exhibit specific cognitive and behavioral problems. Functional MRI has catalyzed insights into brain functional systems and has already been linked with the neuropsychological performance. We aimed to exploit the question of whether resting-state functional MRI can be used as a biomarker for the cognitive outcome assessment of these patients.MethodsWe investigated 13 patients (median age 22.0 years; range 14.9-31.3) after a median interval between surgery and examination of 15.0 years (range 4.2-20.5) and 16 matched controls. All subjects underwent functional 3-Tesla MRI scans in a resting-state condition and battery neuropsychological tests.ResultsPatients showed a significantly increased functional connectivity in the precuneus compared with controls (P < .05) and at the same time impairments in various domains of neuropsychological functioning such as a lower mean Wechsler Intelligenztest für Erwachsene (WIE) IQ percentile (mean [M] = 48.62, SD = 29.14), lower scores in the Trail Making Test (TMT) letter sequencing (M = 49.54, SD = 30.66), worse performance on the WIE subtest Digit Symbol Coding (M = 38.92, SD = 35.29), subtest Symbol Search (M = 40.75, SD = 35.28), and test battery for attentional performance (TAP) divided attention task (M = 783.92, SD = 73.20).ConclusionChildhood cerebellar tumor treated by resection only strongly impacts the development of precuneus/posterior cingulate cortex functional connectivity. Functional MRI has the potential to help deciphering the pathophysiology of cerebellar-related cognitive impairments in these patients and could be an additional tool in their individual assessment and follow-up.

Post-operative cerebellar mutism syndrome: rehabilitation issues

IntroductionTumors of the cerebellum are the most common brain tumors in children. Modern treatment and aggressive surgery have improved the overall survival. Consequently, growing numbers of survivors are at high risk for developing adverse and long-term neurological deficits including deficits of cognition, behavior, speech, and language. Post-operative cerebellar mutism syndrome (pCMS) is a well-known and frequently occurring complication of cerebellar tumor surgery in children. In the acute stage, children with pCMS may show deterioration of cerebellar motor function as well as pyramidal and cranial neuropathies. Most debilitating is the mutism or the severe reduction of speech and a range of neurobehavioral symptoms that may occur. In the long term, children that recover from pCMS continue to have more motor, behavioral, and cognitive problems than children who did not develop pCMS after cerebellar tumor surgery. The severity of these long-term sequelae seems to be related to the length of the mute phase.Aim of this narrative reviewThe impact of pCMS on patients and families cannot be overstated. This contribution aims to discuss the present knowledge on the natural course, recovery, and rehabilitation of children with pCMS. We suggest future priorities in developing rehabilitation programs in order to improve the long-term quality of life and participation of children after cerebellar tumor surgery and after pCMS in particular.

Neuronal correlates of cognitive function in patients with childhood cerebellar tumor lesions.

While it has been shown that cerebellar tumor lesions have an impact on cognitive functions, the extent to which they shape distant neuronal pathways is still largely undescribed. Thus, the present neuroimaging study was designed to investigate different aspects of cognitive function and their neuronal correlates in patients after childhood cerebellar tumor surgery. An alertness task, a working memory task and an incompatibility task were performed by 11 patients after childhood cerebellar tumor surgery and 17 healthy controls. Neuronal correlates as reflected by alterations in functional networks during tasks were assessed using group independent component analysis. We were able to identify eight networks involved during task performance: default mode network, precuneus, anterior salience network, executive control network, visual network, auditory and sensorimotor network and a cerebellar network. For the most ‘basic’ cognitive tasks, a weaker task-modulation of default mode network, left executive control network and the cerebellar network was observed in patients compared to controls. Results for higher-order tasks are in line with a partial restoration of networks responsible for higher-order task execution. Our results provide tentative evidence that the synchronicity of brain activity in patients was at least partially restored in the course of neuroplastic reorganization, particularly for networks related to higher-order cognitive processes. The complex activation patterns underline the importance of testing several cognitive functions to assess the specificity of cognitive deficits and neuronal reorganization processes after brain lesions.

Risk factors for development of postoperative cerebellar mutism syndrome in children after medulloblastoma surgery.

OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis. Tumor type, cerebellar midline location, and brainstem involvement are risk factors for pCMS that have been identified repeatedly, but they do not explain its latent onset. Ambiguous or negative results for other factors, such as hydrocephalus, postoperative meningitis, length of vermian incision, and tumor size, have been reached. The aim of this study was to identify perioperative clinical, radiological, and laboratory factors that also increase risk for the development of pCMS. The focus was on factors that might explain the delayed onset of pCMS and thus might provide a time window for taking precautionary measures to prevent pCMS or reduce its severity. The study was focused specifically on children who had undergone surgery for medulloblastoma. METHODS In this single-center retrospective cohort study, the authors included 71 children with medulloblastoma, 28 of whom developed pCMS after primary resection. Clinical and laboratory data were collected prospectively and analyzed systematically. Variables were included for univariate and multivariate analysis. RESULTS Univariate regression analysis revealed 7 variables that had a significant influence on pCMS onset, namely, tumor size, maximum tumor diameter > 5 cm, tumor infiltration or compression of the brainstem, significantly larger decreases in hemoglobin (p = 0.010) and hematocrit (p = 0.003) in the pCMS group after surgery than in the no-pCMS group, significantly more reported incidents of severe bleeding in the tumor bed during surgery in the pCMS group, preoperative hydrocephalus, and a mean body temperature rise of 0.5°C in the first 4 days after surgery in the pCMS group. Multiple regression analysis revealed that tumor size, tumor infiltration into or compression of the brainstem, and higher mean body temperature in the first 4 postoperative days were independent and highly significant predictors for pCMS. CONCLUSIONS The authors confirmed earlier findings that tumor-associated preoperative conditions, such as a maximum tumor diameter ≥ 5 cm and infiltration into or compression of the brainstem, are associated with a higher risk for the development of pCMS. Most importantly, the authors found that a 0.5°C higher mean body temperature in the first 4 postoperative days increased the odds ratio for the development of pCMS almost 5-fold. These data suggest that an important focus for the prevention of pCMS in children who have undergone medulloblastoma surgery might be rigorous maintenance of normothermia as standard care after surgery.

Transient internuclear ophthalmoparesis associated with mutism following midline cerebellar tumour surgery in an 8-year boy

Abstract Mutism and neurobehaviour symptoms are well known features, which may occur following surgical excision of mass lesion of various histopathologies in the posterior cranial fossa, during the postoperative period. Mutism may be rarely associated with ataxia of eyelid closure and paresis of external ocular muscles. However, internuclear ophthalmoparesis is not reported in association with mutism following posterior cranial fossa surgery. We report an 8-year–boy, who developed internuclear ophthalmoparesis following suboccipital craniectomy for decompression of vermian medulloblastoma. The clinical features, aetiopathogenesis, management of transient internuclear ophthalmoparesis associated with mutism and pertinent literature is reviewed in short.

Lateral medullary infarction masquerading as involvement of spinal cord

Dear Editor, Unilateral sensory impairment below the dermatome usually suggests involvement of the spinal cord. Here, we describe a patient presenting with impaired pain and temperature sensation below the right-side Th8 dermatome due to left lateral medullary infarction. An 86-year-old woman visited our clinic presenting with numbness in her right lower leg. Her past medical history included hypertension, diabetes mellitus, dyslipidemia, hyperthyroidism, and postoperative state after the removal of a hemangioblastoma on the right cerebellum. Her consciousness was clear. Vital signs and general physical examination were normal. Neurological examination revealed deficits in pain and temperature sensation below the Th8 dermatome on the right trunk and leg (Fig. 1a). She showed dysmetria of the right arm and a wide-based gait, but both had been present for years since cerebellar tumor surgery. Motor paralysis or Horner’s syndrome was not seen. We initially suspected that she had involvement of the thoracic spinal cord. However, spinal MRI demonstrated no abnormality in the spine or spinal cord. Brain MRI revealed a small infarction in the left lateral medulla oblongata by diffusion-weighted image (Fig. 1b) and fluid attenuation inversion recovery (Fig. 1c). Magnetic resonance angiography showed an atherosclerotic stenosis at left vertebral artery (Fig. 1d), and high-echoic plaques were found in the bilateral commonand internal-carotid arteries by carotid ultrasonography. Echocardiography and 24-h EEG were normal. The patient was clinically diagnosed with atherothrombotic infarction on the basis of NINDS III classification [1]. Antiplatelet therapy was started subsequently, and the sensory impairment gradually resolved. The lateral spinothalamic tract conveys pain and temperature sensation in the contralateral body and is arranged topographically in the medulla oblongata. Afferents from caudal areas are located in the lateral portions of the tract [2] (Fig. 1e). In our case, the lesion was mostly restricted to the left lateral portion of the spinothalamic tract, affecting sensory fibers conveying superficial sensory information from the sacral, lumbar, and thoracic (to Th8) dermatomes on the right side. Accordingly, the sensory impairment was seen below the right Th8 dermatome. Reports of such patients presenting with sensory deficits in a dermatomal

Auditory-perceptual speech analysis in children with cerebellar tumours: A long-term follow-up study

Mutism and Subsequent Dysarthria (MSD) and the Posterior Fossa Syndrome (PFS) have become well-recognized clinical entities which may develop after resection of cerebellar tumours. However, speech characteristics following a period of mutism have not been documented in much detail. This study carried out a perceptual speech analysis in 24 children and adolescents (of whom 12 became mute in the immediate postoperative phase) 1–12.2 years after cerebellar tumour resection. The most prominent speech deficits in this study were distorted vowels, slow rate, voice tremor, and monopitch. Factors influencing long-term speech disturbances are presence or absence of postoperative PFS, the localisation of the surgical lesion and the type of adjuvant treatment. Long-term speech deficits may be present up to 12 years post-surgery. The speech deficits found in children and adolescents with cerebellar lesions following cerebellar tumour surgery do not necessarily resemble adult speech characteristics of ataxic dysarthria.

Posterior fossa syndrome in adults: A new case and comprehensive survey of the literature

Although the posterior fossa syndrome (PFS) can be considered as an aetiologically heterogeneous condition affecting children and adults, it most often occurs in paediatric patients after cerebellar tumour surgery. In patients with a tumoural aetiology, the syndrome is typically characterised by a short symptom-free postoperative period followed by mutism of variable duration and behavioural and affective changes. More than 200 paediatric cases have been described but reports of adult patients are extremely rare.This paper discusses PFS in adults on the basis of a comprehensive literature survey and describes the pre- and postoperative findings in a new adult patient. In the preoperative phase, cognitive, behavioural and affective abnormalities were identified, matching a diagnosis of cerebellar cognitive affective syndrome (CCAS) (Schmahmann and Sherman, 1998; Schmahmann, 2004). The immediate postoperative course was characterised by prefrontal-like behavioural and affective abnormalities, peduncular hallucinations and confusion evolving to psychosis. Akinetic mutism subsequently developed, lasted for 12 days and then alternated with episodes of diminished responsiveness in which pathological laughing and crying (PLC) occurred. Akinetic mutism resolved after treatment with a non-ergoline dopamine-agonist but CCAS persisted during longitudinal follow-up.From a semiological point of view “relapsing-remitting akinetic mutism” and PLC in our patient might add relevant information to current insights in the clinical expression of the PFS. As evidenced by a close parallelism between single photon emission computed tomography (SPECT) and clinical findings, CCAS as well as PFS seem to reflect functional disruption of the cerebello-cerebral network involved in cognitive, behavioural and affective functions. These findings may indicate that both syndromes share overt semiological resemblances and a common pathophysiological substrate. Consequently, CCAS and PFS may both be regarded as cerebellar-induced clinical conditions showing different aspects of a spectrum that range in degree of severity and symptom duration.

The spectrum of neurobehavioural deficits in the Posterior Fossa Syndrome in children after cerebellar tumour surgery

Introduction The Posterior Fossa Syndrome (PFS) may occur in children after resection of cerebellar tumours. The most common feature is mutism, but also oropharyngeal dyspraxia, emotional lability and neuropsychiatric symptoms occur. We analysed the spectrum of behavioural abnormalities, speech and language characteristics during PFS. Methods In order to identify PFS, all children with a cerebellar tumour admitted to our centre in the study period were prospectively assessed before and after tumour surgery. In the case of PFS, children were systematically followed by means of a standard protocol that included a daily neurological examination and assessment of speech behaviour. Speech was recorded on videotape before and immediately after surgery, and in cases of PFS at as short possible intervals for 4 weeks and subsequently every second week until the recurrence of speech and normalisation of behaviour. Data regarding clinical and behavioural features, duration of symptoms and mode of recovery were collected. Pre- and postoperative MRI data were studied. In 13 children with and two children without PFS a 99mTc-hexamethylpropyleneamine oxime-Single Photon Emission Tomography (SPECT) scan could be performed. Results PFS occurred in 41 of 148 children. During recovery all children were dysarthric, but only in a few speech features specific for cerebellar dysarthria occurred. A significant correlation was found between duration of mutism and severity of neurological symptoms. Significant correlations were also found between duration of mutism and abnormalities on SPECT scans of the left temporal lobe, the left and right basal nuclei, and the right frontal lobe. Conclusions In this study, impairments of higher cognitive functions were observed in the context of PFS. They varied in severity and composition between children with symptoms fitting into the spectrum of the Cerebellar Cognitive Affective Syndrome. SPECT scan findings suggest that these impairments are secondary to supratentorial metabolic hypofunction following cerebellar surgery.

Transient cerebellar eye closure after posterior fossa surgery in a 5-year-old child

Transient cerebellar eye closure (TCES) is a rare complication of cerebellar tumor surgery in children. The pathogenesis of this problem remains unclear, and controversy exists regarding whether it is a purely psychogenic disorder or an organic syndrome. The anatomical substrate for this transient eye closure remains unknown. Most of the cases reported were associated with the syndrome of mutism. We encountered a case of post-operative TCES in a 5-year-old girl with posterior fossa tumor. We are presenting this rare complication with the hope of elucidating further on clinical course of illness and literature review on the possible pathophysiological mechanism.

Transverse Sinus Thrombosis after Posterior Fossa Surgery for Cerebellar Tumor Treated by Endovascular Thrombectomy

Dural sinus thrombosis is a rare complication after posterior fossa surgery, particularly in cerebellar tumour surgery. The authors describe the case of a young male patient who presented a postoperative neurological deterioration due to transverse sinus thrombosis after surgery for cerebellar medulloblastoma. He was treated by mechanical clot thrombectomy using an endovascular catch system technique without anticoagulation therapy. Final angiographic recanalization was obtained. This kind of endoluminal mechanical revascularization is an efficacious method to treat dural sinus thrombosis during perioperative time but speed in diagnosis is crucial for clinical outcome.

Behavioral and affective changes in children and adolescents with chronic cerebellar lesions

The aim of the present study was to investigate if clinically relevant affective or behavioral changes as described in adults in the cerebellar affective syndrome by Schmahmann and Sherman [The cerebellar cognitive affective syndrome, Brain 121 (1998) 561–579] are likely to occur as a long-term sequelae of cerebellar vermis lesions in children. Site and extent of the vermal lesion were defined on the basis of individual 3D-MRI scans and lesion data were correlated with behavioral and affective changes. Affect and behavior were assessed in children after cerebellar tumor surgery by means of experimenter ratings based on the description of the cerebellar affective syndrome and free ratings by the patients and their parents. Twelve children and adolescents with a former cerebellar astrocytoma surgery without subsequent radiation or chemotherapy participated. Detailed analysis of individual 3D-MR images revealed that lesions affected the vermis in nine children. Experimenter ratings according to Schmahmann revealed no relevant problems in patients. In five out of nine patients with vermal affection somewhat increased thoughtful, anxious or aggressive behavior was reported by patients and parents. In conclusion, minor behavioral and affective changes were present in a subset of children with chronic vermal lesions.

138 Long-term cognitive impairment after cerebellar tumour surgery in children

138 Long-term cognitive impairment after cerebellar tumour surgery in children

Alterations of ocular motility in cerebellar pathology. An electro-oculographic study.

Saccadic as well as smooth pursuit movements were studied by means of electro-oculograms in a group of 14 patients affected by cerebellar diseases. Ten patients had cerebellar atrophies, and four had undergone surgery for cerebellar tumors. Loss of gain of the pursuit system and metric alteration of saccades were the most striking abnormalities observed. Dysmetria was shown to be related to the amplitude of the movement and to the sector of the perimeter within which the movement occurs (movements occurring in the more eccentric sector were more disturbed). A tendency to produce saccades slower than normal was noted in patients affected by olivopontocerebellar atrophy. The clinical and pathophysiological significance of this finding is discussed with particular reference to Wadia-Swaami hereditary ataxia.