Karl N. Krecke

Patient Outcomes After Vestibular Schwannoma Management: a Prospective Comparison of Microsurgical Resection and Stereotactic Radiosurgery

OBJECTIVE The best management for patients with small- to medium-sized vestibular schwannomas (VS) is controversial. METHODS : A prospective cohort study of 82 patients with unilateral, unoperated VS less than 3 cm undergoing surgical resection (n = 36) or radiosurgery (n = 46). Patients undergoing resection were younger (48.2 yr versus 53.9 yr, P = 0.03). The groups were similar with regard to hearing loss, associated symptoms, and tumor size. The mean follow-up period was 42 months (range, 12-62 mo). RESULTS Normal facial movement and preservation of serviceable hearing was more frequent in the radiosurgical group at 3 months (P < 0.001), 1 year (P < 0.001), and at the last follow-up examination (P < 0.01) compared with the surgical resection group. Patients undergoing surgical resection had a significant decline in the following subscales of the Health Status Questionnaire 3 months after surgery: physical functioning (P = 0.006), role-physical (P < 0.001), energy/fatigue (P = 0.02), and overall physical component (P = 0.004). Patients in the surgical resection group continued to have a significant decline in the physical functioning (P = 0.04) and bodily pain (P = 0.04) subscales at 1 year and in bodily pain (P = 0.02) at the last follow-up examination. The radiosurgical group had no decline on any component of the Health Status Questionnaire after the procedure. The radiosurgical group had lower mean Dizziness Handicap Inventory scores (16.5 versus 8.4, P = 0.02) at the last follow-up examination. There was no difference in tumor control (100 versus 96%, P = 0.50). CONCLUSION Early outcomes were better for VS patients undergoing stereotactic radiosurgery compared with surgical resection (Level 2 evidence). Unless long-term follow-up evaluation shows frequent tumor progression at currently used radiation doses, radiosurgery should be considered the best management strategy for the majority of VS patients.

CNS aquaporin-4 autoimmunity in children.

Background: In adult patients, autoantibodies targeting the water channel aquaporin-4 (AQP4) are a biomarker for a spectrum of CNS inflammatory demyelinating disorders with predilection for optic nerves and spinal cord (neuromyelitis optica [NMO]). Here we describe the neurologic, serologic, and radiographic findings associated with CNS AQP4 autoimmunity in childhood. Methods: A total of 88 consecutive seropositive children were identified through service evaluation for NMO-IgG. Sera of 75 were tested for coexisting autoantibodies. Clinical information was available for 58. Results: Forty-two patients (73%) were non-Caucasian, and 20 (34%) had African ethnicity. Median age at symptom onset was 12 years (range 4–18). Fifty-seven (98%) had attacks of either optic neuritis (n = 48; 83%) or transverse myelitis (n = 45; 78%), or both. Twenty-six (45%) had episodic cerebral symptoms (encephalopathy, ophthalmoparesis, ataxia, seizures, intractable vomiting, or hiccups). Thirty-eight (68%) had brain MRI abnormalities, predominantly involving periventricular areas (in descending order of frequency): the medulla, supratentorial and infratentorial white matter, midbrain, cerebellum, thalamus, and hypothalamus. Additional autoantibodies were detected in 57 of 75 patients (76%), and 16 of 38 (42%) had a coexisting autoimmune disorder recorded (systemic lupus erythematosus, Sjögren syndrome, juvenile rheumatoid arthritis, Graves disease). Attacks were recurrent in 54 patients (93%; median follow-up, 12 months). Forty-three of 48 patients (90%) had residual disability: 26 (54%) visual impairment and 21 (44%) motor deficits (median Expanded Disability Status Scale 4.0 at 12 months). Conclusions: Aquaporin-4 autoimmunity is a distinctive recurrent and widespread inflammatory CNS disease in children.

Short myelitis lesions in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorders.

IMPORTANCE Short transverse myelitis (STM; <3 vertebral segments) is considered noncharacteristic of neuromyelitis optica (NMO) spectrum disorders (NMOSDs). Nonappreciation of the potential for STM to occur in NMOSD may lead to increased disability from delay in diagnosis and appropriate treatment. OBJECTIVES To determine the frequency of short lesions at the initial myelitis manifestation of NMOSD and to compare the demographic, clinical, and radiological characteristics of aquaporin-4-IgG (AQP4-IgG) seropositive and seronegative STM. DESIGN, SETTING, AND PARTICIPANTS We reviewed the records and images of patients at the Mayo Clinic who were identified as AQP4-IgG positive from 1996 to 2014. Inclusion criteria were first STM episode, magnetic resonance imaging performed 90 days or less from symptom onset, spinal cord T2-hyperintense lesion less than 3 vertebral segments, AQP4-IgG seropositivity, and a final diagnosis of NMO or NMOSD. Patients with an initial longitudinally extensive transverse myelitis were excluded (n = 151). Patients with STM who were seronegative for AQP4-IgG among an Olmsted County population-based cohort of inflammatory demyelinating disorders of the central nervous system were used as a control group. MAIN OUTCOMES AND MEASURES Delay to diagnosis in months, clinical and radiological characteristics, and disability measured by ambulatory status. RESULTS Twenty-five patients who were AQP4-IgG seropositive with an initial STM represented 14% of initial myelitis episodes among patients with NMOSD. The STM episode was defined as the first manifestation of NMOSD in 10 patients (40%) preceded by optic neuritis in 13 patients (52%) and preceded by a nausea and vomiting episode in 2 patients (8%). In comparison with the excluded patients with NMOSD who had an initial longitudinally extensive transverse myelitis, delay to diagnosis/treatment was greater when initial lesions were short (P = .02). In AQP4-IgG-positive STM cases, subsequent myelitis episodes were longitudinally extensive in 92%. Attributes more common in patients with AQP4-IgG-positive STM than in 27 population-based patients with AQP4-IgG-negative STM included the following: nonwhite race/ethnicity; tonic spasms; coexisting autoimmunity; magnetic resonance imaging (central cord lesions, T1 hypointensity, and a brain inconsistent with multiple sclerosis); and cerebrospinal fluid (oligoclonal bands lacking). CONCLUSIONS AND RELEVANCE Short transverse myelitis is not uncommon in NMOSD and, when it is present, delays diagnosis and treatment. Clinical and radiological characteristics identified in this study may help select patients with STM who are at the highest risk for an NMOSD. Short transverse myelitis does not exclude consideration of AQP4-IgG testing or NMOSD diagnosis.

Paraneoplastic isolated myelopathy Clinical course and neuroimaging clues

Objective: To report the clinical phenotype and outcome of isolated paraneoplastic myelopathy. Methods: We systematically reviewed clinical, serologic, and MRI data for 31 patients (20 female) who presented with an isolated myelopathy and coexisting cancer: carcinoma (lung, 9; breast, 7; kidney, 2; thyroid, 2; ovary/endometrium, 2), melanoma (2), or other cancer (3), or a paraneoplastic autoantibody with strong cancer association (amphiphysin–immunoglobulin G [IgG], 9; collapsin response-mediator protein 5–IgG, 9; Purkinje-cell cytoplasmic autoantibody type 1, 2; antineuronal nuclear autoantibody [ANNA]–1, 1; ANNA-3, 1). Results: Of 31 patients who presented with a progressive myelopathy, symptom onset was subacute in 16 (52%). The median age was 62 years. CSF abnormalities included elevated protein (>45 mg/dL), 22; pleocytosis, 15; excess oligoclonal bands (normal <4), 7. MRI cord abnormalities identified in 20 patients were longitudinally extensive (>3 vertebral segments), 14; symmetric tract or gray matter–specific signal abnormality, 15 (enhancing in 13). Myelopathy preceded cancer diagnosis in 18 patients (median interval 12 months; range 2–44). After myelopathy onset, 26 patients underwent oncologic treatment, immunosuppressive treatment (median delay to commencing immunotherapy 9.5 months [range 1–54]), or both; only 8 improved (31%). At last neurologic evaluation (median interval after onset 17 months; range 1–165 months), 16 patients (52%) were wheelchair-dependent (median time from onset to wheelchair 9 months [range 1–21]). Ten patients died after a median of 38 months from symptom onset (range 7–152). Conclusion: Symmetric, longitudinally extensive tract or gray matter–specific changes on spinal MRI should raise suspicion for a paraneoplastic myelopathy. Resulting disability is often severe. Only a minority of patients improve with treatment.

Developing a Radiology Quality and Safety Program: A Primer

Four main areas of quality need to be addressed for a complete quality and safety program in radiology: safety, process improvement, professional outcome assessment, and satisfaction. These areas need to be coordinated by individuals who belong to a quality oversight committee. Management of the data can be facilitated by using a quality scorecard that posts relevant data for each operational group within a department. The ultimate goal is a cultural shift in which all departmental workers assume responsibility for quality and safety improvements and behave consistently with the core values of the organization. A road map for thinking about quality and safety issues in radiology allows all of these areas to be tied together. Four main areas of development are required, each demanding a different skill set and approach.

Intractable nonlesional epilepsy of temporal lobe origin Lateralization by interictal SPECT versus MRI

Article abstract –We performed a retrospective study of 53 consecutive “nonlesional” temporal lobectomy patients to assess the relative utility of MRI versus interictal single-photon emission computed tomography (SPECT) in this patient population. We compared the seizure lateralizing properties of MRI and SPECT using multiple blinded expert reviewers for both SPECT and MRI with a test-retest reviewer paradigm and measurements of hippocampal volume from MRI. The criterion standard for seizure lateralization was satisfactory postoperative seizure control (n = 43). The rate of correct seizure lateralization was significantly greater for MRI than for SPECT (p ≤ 0.01), and the rate of incorrect lateralization was significantly less for MRI than for SPECT. The most accurate MRI measure was hippocampal volume measurements, which correctly lateralized the seizures in 86.0% of cases. The correct lateralization rate for SPECT was 45.4%. The MRI and SPECT studies tended to be noncomplementary with respect to seizure lateralization, and SPECT was likely to give an incorrect or indeterminate result in patients who were not lateralized by MRI. Concordant MRI-EEG lateralization was a strong predictor of satisfactory postoperative seizure control, while no relationship between postoperative seizure control and SPECT findings was present.

Magnetic Resonance Imaging in Cancer-Related Lumbosacral Plexopathy

OBJECTIVE To study the relative utility of computed tomography (CT) and magnetic resonance imaging (MRI) of the lumbosacral plexus in patients with systemic cancer and plexopathy. DESIGN In a retrospective study, we identified all patients encountered at Mayo Clinic Rochester between 1987 and 1993 with a diagnosis of lumbosacral plexopathy, and we selected for analysis those with MRI scans of the plexus (an abnormal finding was not necessary for inclusion) and a clinical and electrophysiologic appearance consistent with a diagnosis of metastatic lumbosacral plexopathy. MATERIAL AND METHODS The study group consisted of 31 patients (20 men and 11 women). The types of tumor were as follows: prostatic, 10 patients; colorectal, 7; bladder, 3; cervical, 3; and other, 8. Eighteen patients had received pelvic radiotherapy before diagnosis of lumbosacral plexopathy. All available MRI scans (in 27 patients) were reviewed blinded; the initial imaging report was used if the actual scans were unavailable (in 4). CT had been done in 22 patients, and results for 16 were available for blinded review. Original reports were available for the other six. RESULTS Direct involvement of the lumbosacral plexus by tumor was evident on 23 MRI studies, and 6 others showed widespread metastatic disease in the region of the plexus. On 13 CT examinations, direct involvement of the lumbosacral plexus by tumor was noted. In four patients, MRI findings were abnormal and CT findings were normal. No patient had abnormal CT findings and normal MRI findings. CONCLUSION In this retrospective review, MRI was more sensitive than CT for diagnosing cancer-induced lumbosacral plexopathy. Thus, use of MRI should be considered in the diagnostic work-up of patients with clinical and electrophysiologic evidence of plexopathy and suspected systemic cancer.

Discriminating long myelitis of neuromyelitis optica from sarcoidosis

To compare longitudinally extensive myelitis in neuromyelitis optica spectrum disorders (NMOSD) and spinal cord sarcoidosis (SCS).

Specific pattern of gadolinium enhancement in spondylotic myelopathy

To highlight a specific under‐recognized radiological feature of spondylotic myelopathy often resulting in misdiagnosis.

Sporadic leucodystrophy with neuroaxonal spheroids: persistence of DWI changes and neurocognitive profiles: a case study

Leucodystrophy with neuroaxonal spheroids (LNS) is rare. There have been fewer than 10 sporadic cases reported, all occurring in the fourth to sixth decades of life. Previously unreported diffusion weighted imaging (DWI) changes on brain imaging in LNS are described as well as the first neurocognitive profile of this disorder in a 24-year-old woman. Neuropsychological testing demonstrated a global cognitive decline, with deficits most representative of a frontal-subcortical dementia. Bright DWI and corresponding dark apparent diffusion coefficient changes were initially mistaken for acute cerebral infarction but then persisted for 19 weeks. Biopsy of a bright DWI lesion showed no evidence of vascular disease and confirmed this rare diagnosis. Given the number of patients with the diagnosis of cerebrovascular disease, supported by DWI findings, we propose other milder cases of LNS may be overlooked.