Kotsenas, Amy L.

Objective To describe an expanded phenotypic spectrum and longitudinal outcome in 256 LGI1‐IgG–seropositive and/or CASPR2‐IgG–seropositive patients. Methods Patients were identified through service neural autoantibody evaluation. Ninety‐five had longitudinal follow‐up (7–456 months; median = 35). Results Among 3,910 patients tested, 196 were LGI1‐IgG positive, 51 were CASPR2‐IgG positive, and 9 were dual positive. Cerebrospinal fluid testing was less sensitive than serum testing, detecting only 24 of 38 (63%) LGI1‐IgG–positive and 5 of 6 (83%) CASPR2‐IgG–positive patients. LGI1‐IgG–positive specimens had higher voltage‐gated potassium channel–IgG immunoprecipitation values (0.33nmol/l, range = 0.02–5.14) than CASPR2‐IgG–positive specimens (0.10nmol/l, range = 0.00–0.45, p < 0.001). Of patients presenting with pain or peripheral nervous system (PNS) manifestations, 39% were LGI1‐IgG seropositive (7% had solely neuropathy or pain). Multivariate analysis identified age as the only significant predictor of central nervous system (CNS) versus PNS involvement (>50 years; odds ratio = 15, p < 0.001). Paroxysmal dizziness spells (PDS), a unique LGI1‐IgG accompaniment (14% of patients), frequently delayed the diagnosis. T2‐mesiotemporal hyperintensity was more common in LGI1‐IgG–positive (41%) than in CASPR2‐IgG–positive patients ( p = 0.033). T1‐bright basal ganglia were confined to LGI1‐IgG–positive patients with faciobrachial–dystonic seizures (9 of 39, 31%). Cancer was found in 44% of LGI1‐IgG/CASPR2‐IgG dual seropositive patients (one‐third thymoma). Response to initial immunotherapy was favorable in 97%; mean modified Rankin score was 3 (range = 1–5) at onset and 1.74 (range = 0–6) at last follow‐up, with 9% having severe refractory disability, 20% being asymptomatic, 28% receiving immunotherapy, and 58% receiving antiepileptic medication. Interpretation Older age is a strong predictor of CNS involvement in patients seropositive for CASPR2‐IgG or LGI1‐IgG. Pain, peripheral manifestations, and stereotypic paroxysmal dizziness spells are common with LGI1‐IgG. Response to initial immunotherapy is often favorable, but some patients remain severely disabled, requiring long‐term immunotherapy and/or antiepileptic medications. Ann Neurol 2017;82:79–92

ABSTRACTPURPOSEInvestigate the relative frequency of nonlesional versus lesional hypertrophic olivary degeneration (HOD) and potential explanations for nonlesional HOD.METHODSOur radiology report database was queried for “HOD” in all head MRI reports between 7/1/2002 and 7/1/2013 and identified 138 patients. Specific absence of HOD or cases with lesions within the dento‐rubro‐olivary pathway (DROP) or extending into the inferior olivary nuclei (ION) were excluded (22 in total). For included patients, 19 locations with the anatomic potential to connect to the ION or DROP were reviewed and grouped into embryological categories, along with the use of the drug metronidazole.RESULTSA total of 51 of 116 (44%) HOD patients did not have a causative lesion in the DROP. The 27/51 (53%) of these patients had progressive ataxia with palatal tremor (PAPT). The 28/51 (55%) patients had one or more lesions outside of the DROP and one of these 28 patients used metronidazole. The remaining 23/51 (45%; 19% of total HOD cohort) patients had no additional lesions in the brain and 6 of these 23 patients did not have a clinical diagnosis of PAPT.CONCLUSIONForty‐four percent of patients with HOD did not demonstrate an MRI‐identifiable lesion in the DROP, and 45% of those patients did not have identifiable brain lesions outside of the DROP. Thus, nearly a fifth of HOD cases may be truly idiopathic. A majority of patients without a DROP lesion had bilateral HOD and two‐thirds were male.

A 67-year-old male presented to the emergency department with concern for accidental aspiration of an aluminum beverage can pull tab. Neck and chest radiographs did not reveal an aspirated foreign body. Despite ongoing complaint of dysgeusia and adamancy of aspiration by the patient, he was discharged to home without recommendation for further follow-up. Seven months later, a computed tomography (CT) scan of the chest performed as part of an unrelated lung cancer work up confirmed the presence of a left mainstem bronchus metallic foreign body compatible with a pull tab. This case report illustrates the poor negative predictive value of radiographs for a suspected aluminum foreign body and demonstrates the superiority of CT for this purpose. In such presentations it is imperative to have a low threshold for performing further diagnostic evaluation with CT due to the relatively high radiolucency of aluminum.

ABSTRACTBACKGROUND AND PURPOSEHypertrophic olivary degeneration (HOD) is an uncommon type of transneuronal degeneration. Case reports and case series described in the literature provide a foundation of our current knowledge of HOD. These reports have described HOD most frequently to be unilateral and occurring in association with lesions in the dentato‐rubro‐olivary pathway. Our purpose was to evaluate the rate of bilateral versus unilateral HOD in a large case series.METHODSA retrospective review was performed to identify patients in which the phrase “hypertrophic olivary degeneration” occurred in the radiology report. A diagnosis of HOD was confirmed on imaging if there was focal hyperintensity on T2‐weighted images confined to either or both inferior olivary nuclei.RESULTSA total of 102 patients had findings consistent with HOD. Of these, 76% had findings bilaterally. In 44%, a lesion could not be identified to explain HOD. Bilateral HOD was common in both lesional and nonlesional group, though more common in the nonlesional group.CONCLUSIONThis study demonstrates that HOD is frequently bilateral. In slightly over 50% of patients with HOD, a lesion can be identified. In just under 50% patients with HOD, a lesion could not be identified and in these cases HOD was present bilaterally in the majority.

ABSTRACTTwo valuable confirmatory MRI findings of acute spinal cord infarct are highlighted and discussed: concomitant vertebral body infarct and ventral cauda equina nerve root enhancement.