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.
Objective:To characterize the clinical features and MRI abnormalities of leucine-rich glioma-inactivated 1 (LGI1)-autoantibody (Ab) faciobrachial dystonic seizures (FBDS).Methods:Forty-eight patients with LGI1-Ab encephalopathy were retrospectively identified by searching our clinical and serologic database from January 1, 2002, to June 1, 2015. Of these, 26 met inclusion criteria for this case series: LGI1-Ab seropositivity and FBDS. In a separate analysis of all 48 patients initially identified, the MRIs of patients with (n = 26) and without (n = 22) FBDS were compared by 2 neuroradiologists blinded to the clinical details.Results:The median age of the 26 included patients was 62.5 years (range 37–78); 65% were men. FBDS involved arm (26), face (22), and leg (12). Ten were previously diagnosed as psychogenic. Ictal EEGs were normal in 20 of 23 assessed. Basal ganglia T1 and T2 signal abnormalities were detected in 11 patients (42%), with excellent agreement between neuroradiologists (κ scores of 0.86 and 0.93, respectively), and included T1 hyperintensity alone (2), T2 hyperintensity alone (1), or both (8). The T1 hyperintensities persisted longer than the T2 hyperintensities (median 11 weeks vs 1 week, p = 0.02). Improvement with immunotherapy (18/18) was more frequent than with antiepileptic medications (10/24). A separate analysis of all 48 patients initially identified with LGI1-Ab encephalopathy showed that basal ganglia MRI abnormalities were present in 11 of 26 with FBDS but not present in those without FBDS (0/22) (p < 0.001). In contrast, mesial temporal MRI abnormalities were less common among those with FBDS (42%) than those without (91%) (p < 0.001).Conclusions:Basal ganglia T1 hyperintensity is a clinically useful MRI biomarker of LGI1-Ab FBDS and suggests a basal ganglia localization.
Under controlled conditions, 1.5-T MRI can be successfully performed in most patients without the need for cochlear implant magnet removal. In nearly all cases, imaging artifact does not impede evaluation of the ipsilateral skull base. Patients should be counseled regarding the risk of internal magnet movement that may occur in up to 15% of cases, even with tight headwrap application. If internal magnet polarity reversal occurs, a trial of reversing the external magnet can be considered. If canting or mild displacement of the internal magnet occurs, an attempt at reseating can be made by applying gentle firm pressure to the scalp over the internal magnet. If conservative measures fail, the magnet should be surgically repositioned to minimize interruption of device use and to prevent scalp complications.
BACKGROUND AND PURPOSE:Autoimmune voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and suggest it in the differential diagnosis because this seizure etiology is likely under-recognized. Our purpose was to characterize MR imaging findings in this patient population.
Although 7-T MRI has recently received approval for use in clinical patient care, there are distinct safety issues associated with this relatively high magnetic field. Forces on metallic implants and radiofrequency power deposition and heating are safety considerations at 7 T. Patient bioeffects such as vertigo, dizziness, false feelings of motion, nausea, nystagmus, magnetophosphenes, and electrogustatory effects are more common and potentially more pronounced at 7 T than at lower field strengths. Herein the authors review safety issues associated with 7-T MRI. The rationale for safety concerns at this field strength are discussed as well as potential approaches to mitigate risk to patients and health care professionals.
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