Long-term P300 in hemispherectomized patients / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>In the years around 1990, in Beijing Tiantan Hospital Affiliated to Capital Medical University many children with infantile hemiplegia and intractable epilepsy were treated with further modified anatomical hemispherectomy. We report the follow up of the first six cases. To make good use of these precious clinical data and make clear their neuropsychological state, we performed neuropsychological and neurophysiological measurements in these patients, who were at a median of 17.8 years after hemispherectomy.</p><p><b>METHODS</b>Oddball task was given to the patients and to a normal control group to collect the peak latency (PL) and peak amplitude (PA) of event-related potentials (ERPs)-P300. The P300 data of the two groups were analyzed and the P300 patterns of the six patients are presented. The baseline characteristics and long-term follow-up of the six hemispherectomized patients, especially the long-term seizure control and cognitive function after surgery, are described.</p><p><b>RESULTS</b>Five patients had no seizures and one was almost seizure-free during the years after surgery. Clear P300 was obtained from every electrode in the patients. Differences of P300 between patients and normal control group had no statistical significance. And the maximum PA was at the site of electrode Pz or Cz which was consistent with that of the control group and with previous findings.</p><p><b>CONCLUSIONS</b>Further modified anatomical hemispherectomy has preferable long-term antiepileptic effects. The P300 results of the patients mean that the basic cognitive function of the patients has no difference from the control group. This reflects the plasticity of the hemisphere to some extent and increases the affirmation of the long-term curative effects of further modified anatomical hemispherectomy from both neuropsychological and neurophysiological aspects.</p>

Motor evoked potential of both upper extremities long term after anatomical hemispherectomy / 中华外科杂志

<p><b>OBJECTIVE</b>To explore the function and motor pathway of remained cerebral hemisphere by studying motor evoked potential of both upper extremities on patients long term after anatomical hemispherectomy.</p><p><b>METHODS</b>Five patients after anatomical hemispherectomy, who were marked 5 dispersive sites on head to perform transcranial magnetic stimulation. Recording motor evoked potential of target muscles (brachioradialis muscle and abductor pollicis brevis) of both upper extremities respectively when muscle resting and contracting.</p><p><b>RESULTS</b>Only affected abductor pollicis brevis of case 2 and only affected brachioradialis muscle of case 4 and 5 recorded motor evoked potential when muscle resting. Motor evoked potential of some cases can be recorded simultaneously in homonymous muscles of both sides when muscle resting or contracting.</p><p><b>CONCLUSIONS</b>There exists motor cortex that controls movement of ipsilateral limbs and also ipsilateral motor pathway of corticospinal connection at patients after anatomical hemispherectomy. It also means that the motor function of affected limbs has potency to recover well after hemispherectomy. The mirror movement after hemispherectomy is possible relate to overlap of both limbs' motor cortex.</p>

Upper limbs motor maps in cortex and plasticity after the anatomical hemispherectomy / 中华外科杂志

<p><b>OBJECTIVE</b>To locate motor functional area of patients who undergone modified anatomical hemispherectomy in order to analysis the plasticity of upper limbs motor.</p><p><b>METHODS</b>The patients who undergone modified anatomical hemispherectomy were performed BOLD sequences, to locate functional cortical areas in their residual brain.</p><p><b>RESULTS</b>6 patients have performed examination of BOLD sequences by 3.0-T MRI.5 of them obtained contralateral upper limb motor areas in their residual brain, and 3 of them obtained ipsilateral and contralateral upper limb motor area map in cortex. The ipsilateral upper limb motor areas in the M1, SMA and posterior parietal cortex.</p><p><b>CONCLUSIONS</b>The patients who undergone modified anatomical hemispherectomy is an excellent model to investigate mechanism of plasticity in the developing brain. Functional magnetic resonance (fMRI) provided fine spatial detail of brain responses, would describe the motor functional area of cortical maps. These patients exist ipsilateral motor areas in their residual mono hemisphere. The study indicated there maybe have somewhat extent of correlation between the surgical procedure and the outcome of neuroplasticity.</p>

A novel Krit-1 mutation in Han family with cerebral cavernous malformation / 中华病理学杂志

<p><b>OBJECTIVE</b>To detect the mutations of Krit-1 gene that cause familial cerebral cavernous malformation (CCM) in the Han ethnic origin.</p><p><b>METHODS</b>The subjects were hospitalized in the Department of Neurosurgery, Tiantan Hospital affiliated to Capital University of Medical Sciences. Two families (A and B) and 8 apparently sporadic individuals affected with CCM were screened for mutations of Krit-1 gene. Members of the family CCM have a wide range in age of onset with seizures, headaches and skin lesions. The gene was screened by PCR amplification of 16 exons and mutation was detected by direct sequencing.</p><p><b>RESULTS</b>In family A samples, analysis of the Krit-1 gene revealed a new point mutation in exon 14 [a heterozygous C to G transition at nucleotide 1 289 (counting from the start codon or nt 2 308 counting from the first nt of the mRNA, aligned according to Gene Bank AF388384)] which predicts the substitution of a premature termination codon for Serine at codon 430 (S430X), belonging a nonsense point mutation. No mutation was identified in one of family A members as well as in any of the sporadic individuals with the exception of a single nucleotide polymorphism.</p><p><b>CONCLUSIONS</b>Report the first family in the Han with CCM having a novel mutation in the CCM1 gene on the continent of Asia. The newly identified mutation creates a premature termination codon and is predicted to produce a truncated Krev1 interaction-trapped 1 protein, KRIT1. This result allows efficient presymptomatic molecular diagnosis.</p>