Creating a Real-World Data, United States Healthcare Claims-Based Adaptation of Kurtzke Functional Systems Scores for Assessing Multiple Sclerosis Severity and Progression.

INTRODUCTION: This article describes the development of a unique mapping of the Kurtzke Functional Systems Scores (KFSS) from International Classification of Diseases, 9th revision, Clinical Modification (ICD-9-CM) codes among multiple sclerosis (MS) patients within a US Integrated Delivery Network (IDN). Valid identification of increasing disability may allow deeper insight into MS progression and possible treatments. METHODS: This cohort study identified MS patients in the IDN, Intermountain Healthcare. Experienced clinicians and informaticists mapped electronic health record ICD-9-CM codes to KFSS components generating a modified Kurtzke Expanded Disability Status Scale (EDSS). Modified EDSS scores were used to assess disability progression by calculating means, medians, ranges, and changes in KFSS and modified EDSS scores. RESULTS: Overall, 608/2960 (20.5%) patients were identified as having MS progression and presented a wide range of scores on the EDSS 10-point scale. The median (range) first and second EDSS scores were 0 (0-6) and 5 (1-8), respectively. The median (range) change from first to second score was 5 (1-7.5). The median first KFSS score for all systems was 0, and the mean differed among components. The highest mean first KFSS score (1.06) was measured for sensory function and lowest (0.12) for cerebellar functions. Of the 544 patients with their first EDSS scores in the ≤ 2.5 group, 75.2% and 15.1% had their second EDSS scores in group 3-5.5 and ≥ 6, respectively. Of the 62 patients with their first EDSS score in the 3-5.5 group, 58.1% had their second EDSS scores in group ≥ 6. CONCLUSION: This innovative mapping technique is a promising method for future comparative effectiveness and safety research of Disease-Modifying Therapy in Real-World Data repositories. Future research to validate and expand on this method in another healthcare database is encouraged.

Postoperative MRI Evaluation of a Radiofrequency Cordotomy Lesion for Intractable Cancer Pain.

BACKGROUND AND PURPOSE: There are limited data on the use of postoperative imaging to evaluate the cordotomy lesion. We aimed to describe the cordotomy lesion by using postoperative MR imaging in patients after percutaneous cordotomy for intractable cancer pain. MATERIALS AND METHODS: Postoperative MR imaging and clinical outcomes were prospectively obtained for 10 patients after percutaneous cordotomy for intractable cancer pain. Area, signal intensity, and location of the lesion were recorded. Clinical outcomes were measured by using the Visual Analog Scale and the Brief Pain Inventory-Short Form, and correlations with MR imaging metrics were evaluated. RESULTS: Ten patients (5 men, 5 women; mean age, 58.5 ± 9.6 years) were included in this study. The cordotomy lesion was hyperintense with central hypointense foci on T2-weighted MR imaging, and it was centered in the anterolateral quadrant at the C1-C2 level. The mean percentage of total cord area lesioned was 24.9% ± 7.9%, and most lesions were centered in the dorsolateral region of the anterolateral quadrant (66% of the anterolateral quadrant). The number of pial penetrations correlated with the percentage of total cord area that was lesioned (r = 0.78; 95% CI, 0.44-0.89; P = .008) and the length of T2-weighted hyperintensity (r = 0.85; 95% CI, 0.54-0.89; P = .002). No significant correlations were found between early clinical outcomes and quantitative MR imaging metrics. CONCLUSIONS: We describe qualitative and quantitative characteristics of a cordotomy lesion on early postoperative MR imaging. The size and length of the lesion on MR imaging correlate with the number of pial penetrations. Larger studies are needed to further investigate the clinical correlates of MR imaging metrics after percutaneous cordotomy.

Impact of airway management strategies on magnetic resonance image quality.

BACKGROUND: Use of general anaesthesia or deep sedation during magnetic resonance imaging (MRI) studies leads to pharyngeal muscle relaxation, often resulting in snoring and subsequent vibrations with head micromotion. Given that MRI is very susceptible to motion, this causes artifacts and image quality degradation. The purpose of our study was to determine the effectiveness of different airway management techniques in overcoming micromotion-induced MRI artifacts. METHODS: After obtaining institutional review board approval, we conducted a retrospective study on the image quality of central nervous system MRI studies in nine patients who had serial MRIs under general anaesthesia. All data were obtained from electronic records. We evaluated the following airway techniques: use of no airway device (NAD); oral, nasal, or supraglottic airway (SGA); or tracheal tube. To assess MRI quality, we developed a scoring system with a combined score ranging from 6 to 30. We used the linear mixed model to account for patient-dependent confounders. RESULTS: We assessed 85 MRI studies from nine patients: 48 NAD, 27 SGA, four oral, four nasal, and two tracheal tube. Arithmetical mean combined scores were 21.6, 27.6, 20.3, 15.3, and 29.5, respectively. The estimated mean combined scores for the NAD and SGA cohorts were 22.0 and 27.3, respectively, showing that SGA use improved the combined score by 5.3 (P<0.0001). CONCLUSIONS: The use of an SGA during MRI studies under general anaesthesia or deep sedation significantly improves image quality.

Ocular and orbital complications of intraarterial cisplatin. A case report.

Despite advances in neurosurgery, radiation oncology, and chemotherapy, the prognosis for glioblastoma multiforme remains poor, with a median survival time of 11-12 months. Cisplatin (cis-diamminedichlorideplatinum II) is one treatment for glioblastoma multiforme. Higher response rates have been achieved by intraarterial (i.a.) infusion than by systemic infusion of this agent. Cisplatin therapy may cause neurologic complications, and i.a. delivery has been reported to cause ocular toxicity. We report a patient who experienced intraorbital and intraocular toxicity following supraophthalmic i.a. injection of cisplatin.

Erythropoietin-augmented isovolemic hemodilution in skull-base surgery. Case report.

Human erythropoietin in concert with intraoperative hemodilution, tumor embolization, and surgical staging was used to manage a red blood cell mass in an anemic Jehovah's Witness patient with a hypervascular meningioma. Erythropoietin (3000 U thrice weekly) and oral iron (1300 mg daily) were given for 1 month prior to surgery, raising the hemoglobin level from 11.8 to 14.1 gm/100 ml. A posterior fossa craniectomy combined with a temporal craniectomy was then performed so that partial petrosectomy, section of the transverse sinus, incision of the tentorium, and exposure of the lesion could be carried out. The first stage of the surgery was terminated immediately prior to tumor mobilization. Isovolemic hemodilution was initiated just before the skin incision. Postoperatively, the hemoglobin concentration dropped to 11.5 gm/100 ml. The erythropoietin dose was doubled and administration of oral iron continued, leading to a hemoglobin level of 14.0 gm/100 ml at 1 month after the first operation. The tumor was embolized using superselective catheterization. The next day, at the second stage of the surgery, the tumor was extirpated, again employing isovolemic hemodilution. By the 4th postoperative day, the hemoglobin level had dropped to 9.4 gm/100 ml. The patient made an uncomplicated recovery. Erythropoietin therapy contributed substantially to the successful outcome of this case. Since erythropoietin has the potential to augment all other forms of autologous banking, its role in elective neurosurgery may become increasingly important in an era of heightened concern about heterologous transfusion.

Cytochrome c oxidase-associated Leigh syndrome: phenotypic features and pathogenetic speculations.

Fourteen new cases of cytochrome oxidase (COX)-associated Leigh syndrome (LS) are combined with 20 reported cases to describe the clinical, laboratory, and radiological features of this devastating metabolic condition. Three clinical stages are identified. Most patients have normal neurological development during the first 8-12 months (stage I). Somatic complaints are common, including chronic diarrhea, recurrent vomiting, anorexia, and decelerating body and head growth. The second stage evolves during late infancy and early childhood when motor regression becomes evident. Eye signs, altered breathing patterns, pyramidal, extrapyramidal, and cerebellar signs emerge and sudden clinical deterioration occurs during intercurrent infectious or metabolic stress. The last stage may extend from 2 to 10 years and is manifested by extreme hypotonia, swallowing difficulties and undernutrition. Feeding assistance is necessary and seizures may occur. The CSF lactate concentration is consistently elevated and MRI abnormalities are seen in the subcortical structures. COX deficiency affects most tissues, but is not always generalized. For example, 3 patients with a cardiomyopathy had normal COX activity in cultured skin fibroblasts. Nearly normal amounts of cross-reacting material are present by ELISA and immunoblot analyses. Parental consanguinity has been found in several families, the hereditary pattern is recessive and males are affected more commonly (2:1). The biomolecular abnormality causing COX deficiency in LS is unknown, but the available evidence implicates a nuclear-encoded protein that affects the structure or the stability of the holoenzyme complex.

MR findings in patients with subacute necrotizing encephalomyelopathy (Leigh syndrome): correlation with biochemical defect.

MR studies were correlated with biochemical results in nine children who presented with lactic acidosis and/or abnormal MR findings in the basal ganglia. Neurologic development was delayed in all nine children. Seven of these patients were diagnosed as having subacute necrotizing encephalomyelopathy (SNE, or Leigh syndrome) on the basis of history, clinical findings, and biochemical studies; of the remaining two, one had congenital lactic acidosis and the other had familial bilateral striatal necrosis with no known biochemical correlate. Although the clinical presentation of these patients was similar, we found distinctive MR abnormalities in characteristic locations in the seven patients with SNE, with or without detectable specific mitochondrial enzyme deficiency in cultured skin fibroblast assays. In our case studies of SNE patients with detectable enzyme deficiency states, defects in pyruvate dehydrogenase complex and cytochrome c oxidase have been found. The MR finding of note in SNE is the remarkably symmetrical involvement, most frequently of the putamen. In our study, lesions were also commonly found in the globus pallidus and the caudate nucleus, but never in the absence of putaminal abnormalities. Other areas of involvement included the paraventricular white matter, corpus callosum, substantia nigra, decussation of superior cerebellar peduncles, periaqueductal region, and brainstem. In patients who present with lactic acidosis and whose MR findings show symmetrical abnormalities in the brain, but with sparing of the putamen, the diagnosis of SNE is in doubt.

MR findings in patients with subacute necrotizing encephalomyelopathy (Leigh syndrome): correlation with biochemical defect.

MR studies were correlated with biochemical results in nine children who presented with lactic acidosis and/or abnormal MR findings in the basal ganglia. Neurologic development was delayed in all nine children. Seven of these patients were diagnosed as having subacute necrotizing encephalomyelopathy (SNE, or Leigh syndrome) on the basis of history, clinical findings, and biochemical studies; of the remaining two, one had congenital lactic acidosis and the other had familial bilateral striatal necrosis with no known biochemical correlate. Although the clinical presentation of these patients was similar, we found distinctive MR abnormalities in characteristic locations in the seven patients with SNE, with or without detectable specific mitochondrial enzyme deficiency in cultured skin fibroblast assays. In our case studies of SNE patients with detectable enzyme deficiency states, defects in pyruvate dehydrogenase complex and cytochrome c oxidase have been found. The MR finding of note in SNE is the remarkably symmetrical involvement, most frequently of the putamen. In our study, lesions were also commonly found in the globus pallidus and the caudate nucleus, but never in the absence of putaminal abnormalities. Other areas of involvement included the paraventricular white matter, corpus callosum, substantia nigra, decussation of superior cerebellar peduncles, periaqueductal region, and brainstem. In patients who present with lactic acidosis and whose MR findings show symmetrical abnormalities in the brain, but with sparing of the putamen, the diagnosis of SNE is in doubt.