Jones, Lyell K.

ABSTRACTIntroduction: Paresis is a long‐recognized complication of herpes zoster, but there has been comparatively little study of zoster‐associated limb paresis (ZALP). Methods: In this study we reviewed 49 Mayo Clinic patients with ZALP. Results: The mean age of onset was 71 years, 67% were men, and the lower limb was affected in 55%. The mean weakness score was 2.0 (0 = normal strength, 4 = plegia). Most patients developed postherpetic neuralgia (PHN, 92% at 1 month and 65% at 3 months), and the average minimum duration of weakness was 193 days. ZALP was caused by radiculopathy (37%), plexopathy (41%), mononeuropathy (14%), and radiculoplexus neuropathy (8%). MRI demonstrated nerve enlargement, T2 signal prolongation, or enhancement in a majority (64%) of affected plexi and peripheral nerves. Conclusions: ZALP is associated with considerable weakness. It typically lasts at least several months, localizes to plexus or peripheral nerve in 63%, and is associated with high rates of PHN. Muscle Nerve 50:177–185, 2014

ABSTRACTIntroduction: Repetitive discharges may be recorded during nerve conduction studies (NCS) or during needle electromyography in a muscle at rest. Repetitive discharges that occur during voluntary activation and are time‐locked to voluntary motor unit potentials (MUP) have not been described. Methods: Retrospective review of motor unit potential induced repetitive discharges (MIRDs) identified in the EMG laboratory. Characteristics of each MIRD, patient demographics, other EMG findings in the same muscle, and electrophysiological diagnosis were analyzed. Results: MIRDs were observed in 15 patients. The morphology and number of spikes and duration of MIRDs varied. The discharges fired at rates of 50–200 Hz. All but 2 patients had EMG findings of a chronic neurogenic disorder. Conclusions: MIRDs are rare iterative discharges time‐locked to a voluntary MUP. The pathophysiology of MIRDs is unclear, but their presence may indicate a chronic neurogenic process. Muscle Nerve 48: 977–979, 2013

ABSTRACTIntroduction: We investigated the clinical, electrophysiological and neural autoantibody characteristics in cramp‐fasciculation syndrome (CFS) patients. Methods: We reviewed Mayo Clinic records from 2000 to 2011 to identify clinically defined CFS patients who underwent neural autoantibody testing. Stored sera of patients who tested positive for antibodies to voltage‐gated potassium channel complex (VGKC complex) were analyzed further for leucine‐rich glioma‐inactivated 1 (LGI1) or contactin‐associated protein‐2 immunoglobulin G (CASPR2‐IgG) antibodies. Results: Thirty‐seven patients were identified. Twelve were seropositive for neural autoantibodies. Clinical manifestations were similar in seropositive and seronegative patients, although central and autonomic neuronal hyperexcitability symptoms were more common in seropositive cases. No patients had a malignancy. Repetitive tibial nerve stimulation at 10 Hz revealed longer afterdischarges in seropositive patients. Two of 7 patients with VGKC‐complex autoimmunity demonstrated LGI1 or CASPR2‐IgG antibodies. Only 2 of 12 seropositive patients required immunotherapy. Conclusions: VGKC‐complex autoimmunity occurs in a minority of CFS patients. Antibody positivity was associated with extramuscular manifestations, typically without malignancy. Target antigens within the VGKC complex remain unknown in most patients. Muscle Nerve 49:351–356, 2014

Objective To report a retrospective analysis of patients with migraine headaches treated with rimabotulinumtoxin B as preventive treatment, investigating an association between clinical responsiveness with migraine directionality and migrainous aura. Background The P hase III R esearch E valuating M igraine P rophylaxis T herapy studies demonstrated onabotulinumtoxin A is effective in the preventive management of chronic migraine headaches. J akubowski et al reported greater response to onabotulinumtoxin A in migraine patients reporting inward‐directed head pain (imploding or ocular) compared with outward‐directed head pain (exploding), suggesting subpopulations of patients may be better candidates for its use. No correlation was found between those reporting migrainous aura and onabotulinumtoxin A responsiveness. Methods One hundred twenty‐eight migraine patients were identified who had received rimabotulinumtoxin B injections over an average of 22 months, or 7 injection cycles. Migraine directionality was reported as inward directed (imploding, n = 72), eye centered (ocular, n = 28), outward directed (exploding, n = 16), and mixed (n = 12). Results One hundred two out of one hundred twenty‐eight patients (80%) improved; of these, 58 (57%) demonstrated a >75% reduction in monthly headache frequency (“>75%‐responders”), 76% of which noted sustained benefits >12 months with repeated injections every 10‐12 weeks. Those reporting ocular‐ and imploding‐directed headaches were significantly more likely to be >75%‐responders, compared with exploding‐ and mixed‐directed headaches ( P < .0025). Patients with ocular‐directed headaches were most likely to be sustained >75%‐responders. Patients reporting migrainous aura were more likely to be >75%‐responders ( P = .0007). Those reporting exploding‐ and mixed‐directed headaches were more likely to be nonresponders ( P < .0001). Conclusions Reported migraine directionality and presence of migrainous aura predict migraine headache responsiveness to rimabotulinumtoxin B injections.

Object Investigators conducting the International Study of Unruptured Intracranial Aneurysms, sponsored by the National Institutes of Health, sought to evaluate predictors of future hemorrhage in patients who had unruptured mirror aneurysms. These paired aneurysms in bilateral arterial positions mirror each other; their natural history is unknown. Methods Centers in the US, Canada, and Europe enrolled patients for prospective assessment of unruptured intracranial aneurysms. Central radiological review confirmed the presence or absence of mirror aneurysms in patients without a history of prior subarachnoid hemorrhage (SAH) (Group 1). Outcome at 1 and 5 years and aneurysm characteristics are compared. Results Of 3120 patients with aneurysms treated in 61 centers, 376 (12%) had mirror aneurysms, which are more common in women than men (82% [n = 308] vs 73% [n = 1992], respectively; p <0.001) and in patients with a family history of aneurysm or SAH (p <0.001). Compared with patients with nonmirror saccular aneurysms, a greater percentage of patients with mirror aneurysms had larger (>10 mm) aneurysms (mean maximum diameter 11.7 vs 10.4 mm, respectively; p <0.001). The most common distribution for mirror aneurysms was the middle cerebral artery (34% [126 patients]) followed by noncavernous internal carotid artery (32% [121]), posterior communicating artery (16% [60]), cavernous internal carotid artery (13% [48]), anterior cerebral artery/anterior communicating artery (3% [13]), and vertebrobasilar circulation (2% [8]). When these patients were compared with patients without mirror aneurysms, no statistically significant differences were found in age (mean age 54 years in both groups), blood pressure, smoking history, or cardiac disease. Aneurysm rupture rates were similar (3.0% for patients with mirror aneurysms vs 2.8% for those without). Conclusions Overall, patients with mirror aneurysms were more likely to be women, to report a family history of aneurysmal SAH, and to have larger aneurysms. The presence of a mirror aneurysm was not an independent predictor of future SAHs.