Jeffrey G. Ojemann, Robert L. Grubb, Michael Kyriakos and Kim B. Baker
✓ This 52-year-old woman developed crystal deposition disease involving the cervical vertebrae. She presented with symptomatic spinal cord compression secondary to extensive calcified lesions in the posterior elements of the cervical spine. Surgical decompression with posterior fusion was performed. Histological examination showed hardened deposits of calcium carbonate involving the soft tissue, and dissolution of the vertebral bone trabeculae. There was no inflammatory response to these deposits. One year postoperatively the patient developed severe pulmonary disease associated with the collagen-vascular disorder, scleroderma (calcinosis, Raynaud's phenomenon, esophageal hypomotility, sclerodactyly, and telangiectasia [CREST] syndrome). Calcium carbonate deposition disease represents an unusual clinical entity that is possibly associated with scleroderma or other collagen-vascular diseases, and it is distinct from ligamentum flavum calcification, calcium pyrophosphate deposition disease, and hydroxyapatite deposition disease.
G. Andrew James, Shanti Prakash Tripathi, Jeffrey G. Ojemann, Robert E. Gross and Daniel L. Drane
Functional neuroimaging has shown that the brain organizes into several independent networks of spontaneously coactivated regions during wakeful rest (resting state). Previous research has suggested that 1 such network, the default mode network (DMN), shows diminished recruitment of the hippocampus with temporal lobe epilepsy (TLE). This work seeks to elucidate how hippocampal recruitment into the DMN varies by hemisphere of epileptogenic focus.
The authors addressed this issue using functional MRI to assess resting-state DMN connectivity in 38 participants (23 control participants, 7 patients with TLE and left-sided epileptogenic foci, and 8 patients with TLE and right-sided foci). Independent component analysis was conducted to identify resting-state brain networks from control participants' data. The DMN was identified and deconstructed into its individual regions of interest (ROIs). The functional connectivity of these ROIs was analyzed both by hemisphere (left vs right) and by laterality to the epileptogenic focus (ipsilateral vs contralateral).
This attempt to replicate previously published methods with this data set showed that patients with left-sided TLE had reduced connectivity between the posterior cingulate (PCC) and both the left (p = 0.012) and right (p < 0.002) hippocampus, while patients with right-sided TLE showed reduced connectivity between the PCC and right hippocampus (p < 0.004). After recoding ROIs by laterality, significantly diminished functional connectivity was observed between the PCC and hippocampus of both hemispheres (ipsilateral hippocampus, p < 0.001; contralateral hippocampus, p = 0.017) in patients with TLE compared with control participants. Regression analyses showed the reduced DMN recruitment of the ipsilateral hippocampus and parahippocampal gyrus (PHG) to be independent of clinical variables including hippocampal sclerosis, seizure frequency, and duration of illness. The graph theory metric of strength (or mean absolute correlation) showed significantly reduced connectivity of the ipsilateral hippocampus and ipsilateral PHG in patients with TLE compared with controls (hippocampus: p = 0.028; PHG: p = 0.021, after correction for false discovery rate). Finally, these hemispheric asymmetries in strength were observed in patients with TLE that corresponded to hemisphere of epileptogenic focus; 87% of patients with TLE had weaker ipsilateral hippocampus strength (compared with the contralateral hippocampus), and 80% of patients had weaker ipsilateral PHG strength.
This study demonstrated that recoding brain regions by the laterality to their epileptogenic focus increases the power of statistical approaches for finding interhemispheric differences in brain function. Using this approach, the authors showed TLE to selectively diminish connectivity of the hippocampus and parahippocampus in the hemisphere of the epileptogenic focus. This approach may prove to be a useful method for determining the seizure onset zone with TLE, and could be broadly applied to other neurological disorders with a lateralized onset.
Robert T. Buckley, Anthony C. Wang, John W. Miller, Edward J. Novotny and Jeffrey G. Ojemann
Laser ablation is a novel, minimally invasive procedure that utilizes MRI-guided thermal energy to treat epileptogenic and other brain lesions. In addition to treatment of mesial temporal lobe epilepsy, laser ablation is increasingly being used to target deep or inoperable lesions, including hypothalamic hamartoma (HH), subependymal giant cell astrocytoma (SEGA), and exophytic intrinsic hypothalamic/third ventricular tumors. The authors reviewed their early institutional experience with these patients to characterize clinical outcomes in patients undergoing this procedure.
A retrospective cohort (n = 12) of patients undergoing laser ablation at a single institution was identified, and clinical and radiographic records were reviewed.
Laser ablation was successfully performed in all patients. No permanent neurological or endocrine complications occurred; 2 (17%) patients developed acute obstructive hydrocephalus or shunt malfunction following treatment. Laser ablation of HH resulted in seizure freedom (Engel Class I) in 67%, with the remaining patients having a clinically significant reduction in seizure frequency of greater than 90% compared with preoperative baseline (Engel Class IIB). Treatment of SEGAs resulted in durable clinical and radiographic tumor control in 2 of 3 cases, with one patient receiving adjuvant everolimus and the other receiving no additional therapy. Palliative ablation of hypothalamic/third ventricular tumors resulted in partial tumor control in 1 of 3 patients.
Early experience suggests that laser ablation is a generally safe, durable, and effective treatment for patients harboring HHs. It also appears effective for local control of SEGAs, especially in combination therapy with everolimus. Its use as a palliative treatment for intrinsic hypothalamic/deep intraventricular tumors was less successful and associated with a higher risk of serious complications. Additional experience and long-term follow-up will be beneficial in further characterizing the effectiveness and risk profile of laser ablation in treating these lesions in comparison with conventional resective surgery or stereotactic radiosurgery.
Jeffrey G. Ojemann, T. S. Park, Robert Komanetsky, Richard A. A. Day and Bruce A. Kaufman
✓ The authors investigated the efficacy of anal sphincter electromyography (EMG) in identifying the lower sacral roots during selective dorsal rhizotomy. In nine children undergoing selective dorsal rhizotomy for cerebral palsy (CP) spasticity, direct electrical stimulation of the L1—S5 dorsal and ventral roots was performed while monitoring EMG responses from the anal sphincter and lower-extremity muscles. Anal sphincter activation was seen with stimulation of lumbosacral roots at many levels. Stimulation of dorsal and ventral roots gave anal sphincter EMG responses in 100% of the dorsal and ventral roots from L-4 and caudally. Only at the L-1 level did a minority of nerve roots have anal sphincter response to stimulation. Patterns of extremity muscle and sphincter activation specific to the S3–5 roots, namely anal sphincter activation without activation of other muscle groups, were found in only five (22%) of 23 roots stimulated. The pattern of stimulation responses in the majority of S3–5 roots indicated that the pathophysiology of lower-extremity spasticity in CP may involve the anal sphincter and does not spare the lower sacral roots. Thus, this study indicates that electrophysiological mapping alone, without anatomical identification, cannot be used to identify the lower sacral roots during selective dorsal rhizotomy for CP spasticity, and it proposes a model for investigation of associated bowel and bladder symptoms.
Robert T. Buckley, Tiffany Morgan, Russell P. Saneto, Jason Barber, Richard G. Ellenbogen and Jeffrey G. Ojemann
Functional hemispherectomy is a well-recognized surgical option for the treatment of unihemispheric medically intractable epilepsy. While the resultant motor deficits are a well-known and expected consequence of the procedure, the impact on other cortical functions has been less well defined. As the cortical control of swallowing would appear to be threatened after hemispherectomy, the authors retrospectively studied a pediatric population that underwent functional hemispherectomy for medically intractable epilepsy to characterize the incidence and severity of dysphagia after surgery.
A retrospective cohort (n = 39) of pediatric patients who underwent hemispherectomy at a single institution was identified, and available clinical records were reviewed. Additionally, the authors examined available MR images for integrity of the thalamus and basal ganglia before and after hemispherectomy. Clinical and video fluoroscopic assessments of speech pathology were reviewed, and the presence, type, and duration of pre- and postoperative dysphagia were recorded.
New-onset, transient dysphagia occurred in 26% of patients after hemispherectomy along with worsening of preexisting dysphagia noted in an additional 15%. Clinical symptoms lasted a median of 19 days. Increased duration of symptoms was seen with late (> 14 days postoperative) pharyngeal swallow dysfunction when compared with oral dysphagia alone. Neonatal stroke as a cause for seizures decreased the likelihood of postoperative dysphagia. There was no association with seizure freedom or postoperative hydrocephalus.
New-onset dysphagia is a frequent and clinically significant consequence of hemispherectomy for intractable epilepsy in pediatric patients. This dysphagia was always self-limited except in those patients in whom preexisting dysphagia was noted.
Jeffrey G. Ojemann
Anthony C. Wang, George M. Ibrahim, Andrew V. Poliakov, Page I. Wang, Aria Fallah, Gary W. Mathern, Robert T. Buckley, Kelly Collins, Alexander G. Weil, Hillary A. Shurtleff, Molly H. Warner, Francisco A. Perez, Dennis W. Shaw, Jason N. Wright, Russell P. Saneto, Edward J. Novotny, Amy Lee, Samuel R. Browd and Jeffrey G. Ojemann
The potential loss of motor function after cerebral hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function.
Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)–DTI maps in 25 patients undergoing functional hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI.
Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661–0.998) and 100% specificity (95% CI 0.715–1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572–0.982) and 100% specificity (95% CI 0.715–1.00). Interpreting the fMR images, the reviewers achieved a Fleiss’ kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01).
Functional hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.