James Sharpe's Contributions to Neurology and Neuro-ophthalmology: A Posthumous Tribute 10 Years On.

Dr. Sharpe was a leading eye movement researcher who had also been the editor of this journal. We wish to mark the 10th anniversary of his death by providing a sense of what he had achieved through some examples of his research.

Electroencephalography Findings in Wrong Way Eyes: Unilateral Hemispheric Dysfunction Supporting Smooth Pursuit Asymmetry Hypothesis.

BACKGROUND: Conjugate horizontal eye deviation away from the side of the lesion, termed Wrong Way Eyes (WWE), is a rare manifestation of supratentorial lesions. The proposed etiologic hypotheses include seizure activity, compression of contralateral horizontal gaze pathways from mass effect or midline shift, and asymmetry of hemispheric smooth pursuit mechanisms. We present neurophysiological evidence that favors the asymmetry of hemispheric smooth pursuit hypothesis. METHODS: Electroencephalography (EEG) was performed in 2 patients with large left hemispheric supratentorial lesions, capturing fluctuating periods of (a) unresponsiveness with WWE and (b) relative alertness without WWE. One patient had 5 days of continuous EEG, and the other routine EEG. RESULTS: Neither patient had seizures. EEG showed normal right hemispheric activity during both unresponsiveness with WWE and alertness without WWE states. By contrast, more severe left hemispheric dysfunction was evident in the WWE state compared with the non-WWE state in both patients. In one patient, during the relatively alert state, right-beating nystagmus was observed, and drift of the eyes away from the side of the lesion was reliably seen to occur on eyelid closure and after ipsiversive volitional saccades. CONCLUSIONS: Seizure activity does not account for WWE. Compression of contralateral horizontal gaze pathways is also unlikely to account for WWE as that hypothetical mechanism should produce EEG abnormalities over the nonlesioned hemisphere, which were not seen. The findings suggest instead that a single dysfunctional hemisphere is sufficient to produce WWE. The repeated rightward drift of the eyes and nystagmus seen in one patient during relative alertness, and the observation of unilateral hemispheric dysfunction on EEG during unresponsiveness with WWE in both patients supports the idea that an imbalance of smooth pursuit mechanisms is most likely to account for this rare phenomenon.

Multivariable prediction model for suspected giant cell arteritis: development and validation.

PURPOSE: To develop and validate a diagnostic prediction model for patients with suspected giant cell arteritis (GCA). METHODS: A retrospective review of records of consecutive adult patients undergoing temporal artery biopsy (TABx) for suspected GCA was conducted at seven university centers. The pathologic diagnosis was considered the final diagnosis. The predictor variables were age, gender, new onset headache, clinical temporal artery abnormality, jaw claudication, ischemic vision loss (VL), diplopia, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and platelet level. Multiple imputation was performed for missing data. Logistic regression was used to compare our models with the non-histologic American College of Rheumatology (ACR) GCA classification criteria. Internal validation was performed with 10-fold cross validation and bootstrap techniques. External validation was performed by geographic site. RESULTS: There were 530 complete TABx records: 397 were negative and 133 positive for GCA. Age, jaw claudication, VL, platelets, and log CRP were statistically significant predictors of positive TABx, whereas ESR, gender, headache, and temporal artery abnormality were not. The parsimonious model had a cross-validated bootstrap area under the receiver operating characteristic curve (AUROC) of 0.810 (95% CI =0.766-0.854), geographic external validation AUROC's in the range of 0.75-0.85, calibration pH-L of 0.812, sensitivity of 43.6%, and specificity of 95.2%, which outperformed the ACR criteria. CONCLUSION: Our prediction rule with calculator and nomogram aids in the triage of patients with suspected GCA and may decrease the need for TABx in select low-score at-risk subjects. However, misclassification remains a concern.

A case report of cavernous sinus syndrome in a patient with Takayasu's arteritis.

Neurologists must entertain a broad differential diagnosis when considering a patient with cavernous sinus syndrome, including neoplasm, trauma, vascular causes, inflammatory processes, and infections. We report the case of a 37-year-old woman initially diagnosed with cavernous sinus syndrome, where subsequent investigations revealed findings of Takayasu's arteritis, a large vessel vasculitis. The patient also tested positive for perinuclear antineutrophil cytoplasmic antibodies, suggesting the possibility of a vasculitic spectrum disorder although no clinical features of Wegener's granulomatosis were present. Criteria for Takayasu's arteritis and its protean neurologic manifestations are reviewed. This case highlights the spectrum of vasculitic conditions that may be associated with cavernous sinus inflammation.