Two Different Manifestations of Neonatal Vascular Injury: Dyke-Davidoff-Masson Syndrome and Crossed Cerebellar Atrophy.

Dyke-Davidoff-Masson syndrome (DDMS) was first described in 1933 as a clinical condition characterized by hemiatrophy, hyperpneumatization of paranasal sinuses, contralateral hemiparesis, facial asymmetry, seizures, and mental retardation.1 DDMS can be of 2 types: congenital and acquired. The congenital type can be caused by various conditions experienced during fetal or early childhood development, including ischemia, infarction, trauma, infections, and hemorrhage. The acquired type is mostly associated with hemorrhage, trauma, and infections experienced after 1 month of age. DDMS can manifest alone or can be accompanied by crossed cerebellar atrophy (CCA) which is a newly discovered radiological marker characterized by prominent cortical sulci and loss of cerebellar parenchyma. The congenital type of DDMS is known to be accompanied by ipsilateral cerebellar atrophy and the acquired type is known to be accompanied by contralateral cerebellar atrophy.2,3 Supratentorial events may lead to destruction in the cortico-ponto-cerebellar pathways, mostly in the contralateral side of the body (80%) due to decussation.4 In this report, we present 2 cases of DDMS accompanied by CCA to emphasize the possibility that the DDMS cases with severe intrauterine hemorrhage can be accompanied by contralateral CCA and migratory abnormalities rather than ipsilateral CCA and clinical survey.

Feasibility study of open inguinal hernia repair using mesh plug by residents.

BACKGRAUND/OBJECTIVE: Inguinal hernia repair by mesh-plug (MP) is one of the most common general surgeries, and even residents can perform it. The aim of this study was to investigate the postoperative outcome of MP repair by residents and risk factors related to the recurrence. METHODS: This study included 658 patients underwent MP repair for inguinal hernia. We compared short- and long-term outcomes of the MP repair by residents who were postgraduate year two with those by non-residents. Late complications were investigated via questionnaire. RESULTS: Among the patients, 206 patients (31%) underwent MP repair by residents, and the other 452 patients (69%) by non-residents. Operative time was significantly longer in the resident group (63 vs. 58 min, P = 0.004). Incidence of short- and long-term complications was not significantly different. The 3-year recurrence rate was significantly higher in the resident group (4.1 vs. 0.9%, P = 0.003). By multivariate analysis, independent perioperative risk factors related to recurrence were surgery by residents (Odds ratio 3.42, 95% CI 1.34-8.76, p = 0.010) and direct hernia (Odds ratio 7.69, 95% CI 2.83-20.83, p < 0.001). CONCLUSION: The MP repair by residents and direct hernia were risk factors related to recurrence. Surgeons should provide very careful guidance to residents especially for direct hernia.

Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients.

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.

Electrostimulation for Refractory Epilepsy: A Review.

Epilepsy is a neurological disorder that has been diagnosed in approximately 1% of the world's population. In North America alone, more than 3 million individuals suffer from epilepsy. Antiepileptic drugs are not fully effective in some patients, and most drugs have adverse side effects. Recently, several stimulation techniques (responsive neural, vagal nerve, transcranial magnetic, and deep brain) have been used as adjunct therapies to treat medically refractive seizures. Since its Food and Drug Administration approval in 2013, responsive neural stimulation (RNS), a closed-loop electrical stimulation system, has emerged as a potential therapeutic alternative to treat patients with epilepsy (PWE). RNS consists of a cranially implantable neurostimulator that sends electrical pulses using depth electrodes to epileptic foci/focus after the device senses irregular electrical activity, thus avoiding the onset of a seizure. In a long-term study that lasted 7 yr and involved more than 245 patients using RNS, results showed that16% of patients were seizure free, 60% had 50% or greater seizure reduction, and 84% had some improvement. Quality of life improved in 44% of the patients by the end of the second year. There is a need for more, larger, well-designed, randomized, controlled trials to validate and optimize efficacy and safety of invasive intracranial neurostimulation treatments in PWE. This article highlights the effects of treating patients with medically refractive seizures using RNS.

Role of cortical dysplasia in epileptogenesis following prolonged febrile seizure.

PURPOSE: Hippocampal sclerosis, characterized by prominent neuronal loss and reactive gliosis, is the most common pathology in human temporal lobe epilepsy (TLE). Although prolonged febrile convulsion (FC) is a risk factor of TLE, it is not clear whether FC provokes hippocampal sclerosis and subsequent TLE. Given that underlying brain lesions, such as cortical dysplasia (CD), in the immature brain predispose patients to FC, CD may link FC and TLE. However, the role of CD in epileptogenesis after FC is also unclear. Here, we investigated whether inborn CD increases the risk of later epilepsy induced by prolonged FC using a rat model. METHODS: Experimental CD was induced by in utero exposure of methylazoxymethanol (MAM). Rat pups from MAM-treated or control rats were then subjected to prolonged FC. We examined morphologic changes in the hippocampi with respect to neuronal loss, reactive gliosis, and synaptogenesis, and evaluated spontaneous recurrent seizures (SRS) by long-term video-EEG (electroencephalography). RESULTS: The MAM+FC group had a significantly lower hippocampal neuronal density in the CA1 and dentate hilus than other control groups. A robust increase in glial cells and synaptic reorganization was also detected in the MAM+FC groups. Furthermore, later SRS occurred in all rats in the MAM+FC group and in 50% and 25% of the rats in the FC-only and MAM-only group, respectively. The frequency and total duration of SRS was highest in the MAM+FC group. DISCUSSION: Our results suggest that preexisting CD in the immature brain augments the proepileptogenic effects of prolonged FC, leading to TLE.

Clinical and electroencephalographic characteristics of children with febrile seizures plus.

OBJECTIVES: Febrile seizures plus (FS+) are attracting attention for their corresponding genetic abnormalities, and are defined as febrile seizures (FS) continuing beyond 6 years of age (late FS) or those associated with afebrile seizures. We tried to elucidate their clinical and EEG characteristics as compared with those of children having only FS. SUBJECTS AND METHODS: We reviewed clinical records in a pediatric neurology clinic to identify 31 patients with FS+ (group FS+) and 51 with only FS (group FS). Their family history of seizures, clinical features and EEG findings were compared. RESULTS: A family history of seizures was noted in 14 patients (45.2%) of group FS+ and in 24 (47.1%) of group FS. In group FS+, 19 patients had late FS, 11 had afebrile seizures, and the remaining one had both types of seizures. Two patients had seizures induced by TV/video-game as well, and another suffered from absences. Epileptic EEG abnormalities, which included diffuse spike-waves and focal spikes, were noted in 13 patients (41.9%) of group FS+ and 12 (23.5%) of group FS. CONCLUSIONS: The clinical and EEG characteristics of the children having FS+ were diverse, without significant differences from those with FS except for the seizures types.