Anatomical, physiological and functional neuroimaging studies suggest that the cerebellum participates in the organization of higher order function, but there are very few descriptions of clinically relevant cases that address this possibility. We performed neurological examinations, bedside mental state tests, neuropsychological studies and anatomical neuroimaging on 20 patients with diseases confined to the cerebellum, and evaluated the nature and severity of the changes in neurological and mental function. Behavioural changes were clinically prominent in patients with lesions involving the posterior lobe of the cerebellum and the vermis, and in some cases they were the most noticeable aspects of the presentation. These changes were characterized by: impairment of executive functions such as planning, set-shifting, verbal fluency, abstract reasoning and working memory; difficulties with spatial cognition including visual-spatial organization and memory; personality change with blunting of affect or disinhibited and inappropriate behaviour; and language deficits including agrammatism and dysprosodia. Lesions of the anterior lobe of the cerebellum produced only minor changes in executive and visual-spatial functions. We have called this newly defined clinical entity the 'cerebellar cognitive affective syndrome'. The constellation of deficits is suggestive of disruption of the cerebellar modulation of neural circuits that link prefrontal, posterior parietal, superior temporal and limbic cortices with the cerebellum.

/pdf/the-cerebellar-cognitive-affective-syndrome-2qlts0j1w1.pdf

The cerebellar cognitive affective syndrome.

Malignant gliomas are the most common and deadly brain tumors. Nevertheless, survival for patients with glioblastoma, the most aggressive glioma, although individually variable, has improved from an average of 10 months to 14 months after diagnosis in the last 5 years due to improvements in the standard of care. Radiotherapy has been of key importance to the treatment of these lesions for decades, and the ability to focus the beam and tailor it to the irregular contours of brain tumors and minimize the dose to nearby critical structures with intensitymodulated or image-guided techniques has improved greatly. Temozolomide, an alkylating agent with simple oral administration and a favorable toxicity profile, is used in conjunction with and after radiotherapy. Newer surgical techniques, such as fluorescence-guided resection and neuroendoscopic approaches, have become important in the management of malignant gliomas. Furthermore, new discoveries are being made in basic and translational research, which are likely to improve this situation further in the next 10 years. These include agents that block 1 or more of the disordered tumor proliferation signaling pathways, and that overcome resistance to already existing treatments. Targeted therapies such as antiangiogenic therapy with antivascular endothelial growth factor antibodies (bevacizumab) are finding their way into clinical practice. Large-scale research efforts are ongoing to provide a comprehensive understanding of all the genetic alterations and gene expression changes underlying glioma formation. These have already refined the classification of glioblastoma into 4 distinct molecular entities that may lead to different treatment regimens. The role of cancer stem-like cells is another area of active investigation. There is definite hope that by 2020, new cocktails of drugs will be available to target the key molecular pathways involved in gliomas and reduce their mortality and morbidity, a positive development for patients, their families, and medical professionals alike. CA Cancer J Clin 2010;60:166-193. © 2010 American Cancer Society, Inc.

https://onlinelibrary.wiley.com/doi/pdf/10.3322/caac.20069

Exciting New Advances in Neuro-Oncology The Avenue to a Cure for Malignant Glioma

Status epilepticus (SE) treatment strategies vary substantially from one institution to another due to the lack of data to support one treatment over another. To provide guidance for the acute treatment of SE in critically ill patients, the Neurocritical Care Society organized a writing committee to evaluate the literature and develop an evidence-based and expert consensus practice guideline. Literature searches were conducted using PubMed and studies meeting the criteria established by the writing committee were evaluated. Recommendations were developed based on the literature using standardized assessment methods from the American Heart Association and Grading of Recommendations Assessment, Develop- ment, and Evaluation systems, as well as expert opinion when sufficient data were lacking.

/pdf/guidelines-for-the-evaluation-and-management-of-status-3h3cqurhgk.pdf

Guidelines for the Evaluation and Management of Status Epilepticus

A report of two cases

The cerebellum and cognition.

U Because skeletal dysplasias are primary disorders of bone, they have not been commonly understood as neurosurgical diseases. Nevertheless, neurosurgical complications are commonly encountered in many cases of dysplasia syndromes. The authors present two cases of skeletal dysplasia that caused overt instability of the cervical spine. One patient with a diagnosis of Gorham disease of the cervical spine was treated with prolonged fixation in a halo brace after an initial attempt at instrumentation with a posterior occiput‐C4 fusion. The other patient, who at birth was identified to have camptomelic dysplasia, has been treated conservatively from the outset. Although these two patients presented with different disorders—in one patient adequate mature bone never formed and in the other patient progressive bone loss became apparent after a seemingly normal initial development—these cases demonstrate unequivocally that surgical options for fusion are ultimately limited by the quality of the underlying bone. In patients in whom the bone itself is inadequate for use as a substrate for fusion, there are currently limited treatment options. Future improvements in our understanding of chondrogenesis and ossification may lead to the design of superior methods of encouraging fusion in these patients; however, at the present time, long-term maintenance in a halo brace may, in fact, be the only treatment.

/pdf/report-of-two-cases-and-review-of-the-literature-12jmr5r1y4.pdf

Report of two cases and review of the literature

Pseudotumor cerebri (PTC), or idiopathic intracranial hypertension, is a syndrome associated with multiple clinical conditions. We hypothesize that most if not all etiologies result in an increase in intracranial venous pressure as a final common pathway. We studied 10 patients with PTC. Five had dural venous outflow obstruction as demonstrated by venography, and the five remaining patients had normal venous anatomy. Pressure measurements, made during venography in eight patients, all showed elevated pressures. Pressure measurements in the superior sagittal sinus ranged from 13 to 24 mm Hg (mean, 16.6 mm HG). Patients with obstruction tended to have a high pressure gradient across the stenotic segment. Five patients with normal dural venous anatomy had elevated right atrial pressures (range, 6 to 22 mm Hg; mean, 11.8 mm Hg), which were transmitted up to the intracranial venous sinuses. Endovascular techniques, including angioplasty and infusion of thrombolytic agents in some cases, improved outlet obstruction from a hemodynamic perspective but were ineffective in consistently and reliably alleviating the clinical manifestations of PTC. Patients in both groups tended to respond well to conventional CSF diversion procedures. Our study suggests that elevated intracranial venous pressure may be a universal mechanism in PTC of different etiologies. This elevated venous pressure leads to elevation in CSF and intracranial pressure by resisting CSF absorption. Although the mechanism leading to venous hypertension in the presence of outflow obstruction is obvious, the etiology of increased intracranial and central systemic venous pressure in PTC remains obscure.

Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies

A review of 122 pediatric cases of vertebral column and spinal cord injuries is presented. These relatively uncommon injuries can be characterized by four distinct injury patterns: fracture only, fracture with subluxation, subluxation only, and spinal cord injury without radiographic abnormality. The immature pediatric spine has several anatomical and biomechanical features that distinguish it from the mature adolescent spine and, accordingly, the frequency of the injury type, the level of spine injury, and the incidence of neurological compromise were found to vary with the age of the patient. Follow-up data were obtained in 93% of the cases (median duration 44 months). No patient was made worse by treatment, 89% of the patients with incomplete myelopathy on admission were improved on their last examination, and 20% of the patients with a complete myelopathy had evidence of significant recovery of function. The authors conclude that the outcome after pediatric spinal trauma is good.

Pediatric spinal trauma: Review of 122 cases of spinal cord and vertebral column injuries

t,- A review of 122 pediatric cases of vertebral column and spinal cord injuries is presented. These relatively uncommon injuries can be characterized by four distinct injury patterns: fracture only, fracture with subluxation, subluxation only, and spinal cord injury without radiographic abnormality. The immature pediatric spine has several anatomical and biomechanical features that distinguish it from the mature adolescent spine and, accordingly, the frequency of the injury type, the level of spine injury, and the incidence of neurological compromise were found to vary with the age of the patient. Follow-up data were obtained in 93% of the cases (median duration 44 months). No patient was made worse by treatment, 89% of the patients with incomplete myelopathy on admission were improved on their last examination, and 20% of the patients with a complete myelopathy had evidence of significant recovery of function. The authors conclude that the outcome after pediatric spinal trauma is good.

Review of 122 cases of spinal cord and vertebral column injuries

• Acute bilateral damage to large areas of both cerebellar hemispheres including the dentate nuclei led to temporary loss of speech in six children. In each case muteness was unassociated with motor paralysis, loss of higher cognitive functions, or cranial nerve dysfunction. Muteness lasted one to three months. All patients were severely dysarthric during recovery. We conclude that transient muteness may result from acute bilateral cerebellar injury.

Harold L. Rekate

Papers

Report of two cases and review of the literature

Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies

Pediatric spinal trauma: Review of 122 cases of spinal cord and vertebral column injuries

Review of 122 cases of spinal cord and vertebral column injuries

Muteness of Cerebellar Origin