Ten-Year Follow-Up of Transcranial Magnetic Stimulation Study in a Patient With Congenital Mirror Movements: A Case Report

Most studies concerning congenital mirror movements (CMMs) have been focused on the motor organization in the distal hand muscles exclusively. To the best of our knowledge, there is no data on motor organization pattern of lower extremities, and a scarcity of data on the significance of forearm and arm muscles in CMMs. Here, we describe the case of a 19-year-old boy presenting mirror movements. In these terms, a 10-year transcranial magnetic stimulation study demonstrated that the motor organization pattern of the arm muscles was different from that of distal hand and forearm muscles even in the same upper extremity, and that the lower extremities showed the same pathways as healthy children. Moreover, in this case, an ipsilateral motor evoked potentials (MEPs) for distal hand muscles increased in amplitude with age, even though the intensity of mirror movements decreased. In the arm muscles, however, it was concluded that the contralateral MEPs increased in amplitude with age.

Pyramidal and Proprioceptive Dysfunctions as the Main Neurologic Features in Symptomatic Type I Chiari Malformation Patients

Objective Broader access to magnetic resonance imaging (MRI) has increased the diagnosis of tonsillar ectopia, with most of these patients being asymptomatic. The early diagnosis and treatment of type I Chiari malformation (CM I) patients has impact on the prognosis. This study supplements information about the neurologic exam of symptomatic patients with CM I. Methods The sample was composed of 32 symptomatic patients with CM I diagnosed by a combination of tonsil herniation of more than 5 mm below the magnum foramen (observed in the sagittal T2 MRI) and at least one of the following alterations: intractable occipital headache, ataxia, upper or lower motor neuron impairment, sensitivity deficits (superficial and deep) or lower cranial nerves disorders. Results Occipital headache was the most frequent symptom (53.12%). During the physical exam, the most common dysfunctions were those from the pyramidal system (96.87%), followed by posterior cord syndrome (87.5%). Discussion In this study, patients became symptomatic around the fifth decade of life, which is compatible with previous descriptions. Patients withmore than 2 years of evolution have worse responses to treatment. Occipital headache, symptoms in the upper limbs, gait and proprioceptive disorders are common findings in patients with CM I. Conclusion Deep tendinous reflexes and proprioception disorders were the main neurologic features found in symptomatic CM I patients.

A Case Report Hemiparesis Caused by Ipsilateral Middle Cerebral Artery Infarction

As the corticospinal tract crosses at the medulla, supratentorial stroke generally influences the opposite extremity. However, new incidences of hemiparesis might occur in the ipsilateral brain if there was a previous infarction in the opposite brain or a congenital structural abnormality. The occurrence of ipsilateral hemiparesis after cerebral infarction is very rare; however, we report here the case of a patient who developed right hemiparesis that was identified as acute right middle cerebral artery infarction.

Hiperintensidad de tracto corticoespinal en resonancia magnética nuclear como signo de lesión de neurona motora superior en esclerosis lateral amiotrófica, reporte de caso y revisión de literatura / High-intensity signal in the corticospinal tract in MRI as a sign of upper motor neuron lesion in amyotrophic lateral sclerosis: Case report and literature review

RESUMEN La esclerosis lateral amiotrófica (ELA) es una enfermedad neurodegenerativa que compromete principalmente el sistema motor. Puede ser adquirida de manera esporádica o hereditaria y presenta una incidencia que varía entre 1.5 a 2.7 casos por 100.000 personas/año, con una mediana de supervivencia de 2 a 4 años. Esta enfermedad está caracterizada por compromiso de ambas motoneuronas, superior e inferior y sus manifestaciones clínicas pueden variar desde leve debilidad muscular en etapas tempranas, hasta falla respiratoria asociada en etapas tardías. El diagnóstico es esencialmente clínico y se hace de acuerdo a los criterios revisados de El Escorial para el diagnóstico de Esclerosis Lateral Amiotrófica (ELA). A pesar de esto, el diagnóstico de ELA en etapas tempranas sigue siendo un reto, lo que retrasa su identificación. Por esta razón surge el interés por identificar nuevos biomarcadores que ayuden al diagnóstico más temprano de la entidad. A continuación se presentará el caso de una mujer de 53 años, a la cual se le realiza un diagnóstico de ELA y en la que se encuentra como hallazgo de resonancia magnética cerebral (RM) cerebral, hiperintensidad bilateral del tracto corticoespinal. Discutiremos en este caso la importancia de los hallazgos en neuroimagen como biomarcador de daño en la enfermedad.

SUMMARY Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that mainly involves the motor system. It may be sporadic or hereditary, and has an incidence ranging from 1.5 to 2.7 cases per 100,000 persons / year, with a median survival of 2 to 4 years. This disease is characterized by damage of both upper and lower motor neurons, and its clinical manifestations can vary from mild muscle weakness in early stages, to respiratory failure associated in later stages. The diagnosis is based on clinical findings and is made according to the revised El Escorial criteria. Although, the diagnosis of ALS remains a challenge in the early stages and delays it's identification. For this reason, interest to identify new biomarkers that help earlier identification and monitoring of the progression of the disease are being studied. Authors present a case of a woman of 53 years old with a clinical diagnosis of ALS and evidence of corticospinal tract high intensity signal on brain MRI. We will discuss the relevance of brain imaging findings as a biomarker of upper motoneuron damage in ALS.

Diagnostic accuracy of diffusion tensor imaging in amyotrophic lateral sclerosis / 中华神经科杂志

Objective To evaluate the diagnostic accuracy of diffusion tensor imaging (DTI) of corticospinal tract in amyotrophic lateral sclerosis (ALS) and find optimal testing strategies and optimal cutoff values of DTI indices for individual patient discrimination.Methods Thirty-three ALS patients and 34 healthy controls,collected at Peking Union Medical College Hospital from June 2004 through July 2005,undergoing brain DTI studies and fractional anisotropy (FA) examinations along the corticospinal tract,were analyzed by receiver operating characteristic (ROC) curves.Results Compared with the controls,ALS group had significantly decreased FA values in subcortical white matter of the precentral gyrus,the posterior limb of the internal capsule and the cerebral peduncle.In ROC analysis,the average FA value of the former two positions showed the best performance with an area under the curve of 0.917,an optimal cut-off value of 0.604,a sensitivity of 0.759 and a specificity of 0.912.The corresponding data for the average FA of all the three positions and each single position were listed as follows:average of three 0.914,0.648,0.759,0.912; precentral gyrus 0.875,0.509,0.733,0.824; internal capsule 0.845,0.692,0.656,0.941 ; and cerebral peduncle 0.752,0.742,0.656,0.735.Conclusions FA values of the corticospinal tract have a good accuracy in detecting upper motor neuron involvement in ALS.Precentral gyrus and posterior limb of the internal capsule and the average FA values of these two positions were suggested as the preferred testing places and DTI indices for clinical use.

Osmotic Demyelination Syndrome: Why Should Hypernatremia be Corrected Slowly : A Case Report.

Aim: Our aim is to present a case of hypernatremia which has led on to a flaccid quadriparesis due to brain stem demyelination. Rapid correction of hypernatremia as a cause for pyramidal tract demyelination is not documented in the literature. Presentation of Case: A 53 year old male was brought to the emergency services with suspected stroke. He was treated with intravenous mannitol and oral glycerine from the primary health centre. We detected hypoglycemia (blood sugarwas 50mg/dl-Ref range: ≤70mg%) and dextrose was given intravenously. Subsequently the patient went into a hypernatremic state with serum sodium 170milli equivalents /liter which was corrected rapidly. This was corrected over 48 hours to 140milli equalents/litre. The rate of correction exceeded 0.62millimols/liter/hour (Ideal: 0.5 mmol/L/h). On the 6th day the patient developed acute quadriparesis. Magnetic resonance imaging (MRI) of brain revealed bilateral symmetric demyelination of the corticospinal tracts. Over six months the neurological deficit improved with complete resolution of the changes in previous Discussion: Osmotic Demyelination Syndrome (ODS) has been a recognized complication of rapid correction of hyponatremia. Experiments in animals and clinical experience suggest that correction of chronic hyponatremia should be kept at a slow rate to combat this complication. The characteristic sites include pons and basal ganglia. Such a complication has not been described due to rapid correction of hypernatremia.This is probably the first case report in the literature where acute onset of quadriparesis resulted from demyelination of the pyramidal tract consequent to a rapid correction of hypernatremia. We had to wait about 6 months for the patient to obtain a complete functional recovery and the neuro imaging was repeated after 6 months to confirm the disappearance of the initial findings thus implicating rapid correction of hypernatremia as the cause of his morbidity. Conclusion: This is the first time extrapontine reversible myelinolysis due to rapid correction of hypernatremia has been documented. To prevent this potentially fatal complication it will be prudent if hypernatremia is corrected slowly.

Esclerosis Lateral Amiotrófica. Experiencia quirúrgica en 13 pacientes / Amyotrophic Lateral Sclerosis. Surgical experience in 13 patients

Introducción: En agosto del 2009 operamos el primer paciente con esclerosis lateral amiotrófica (ELA). Sin embargo, hasta la fecha, todo los investigadores informan que no hay cura para esta enfermedad. Objetivo: Demostrar que la ELA puede ser detenida y mejorada mediante un transplante de epiplón. Material y Método: Presentamos a 13 pacientes con formas bulbar y espinal de ELA. Durante la cirugía encontramos: 1) variantes anatómicas del segmento V4 de las arterias vertebrales, 2) aterosclerosis moderada o severa en ambos segmentos V4, 3) algunas arterias circunflejas originadas desde las arterias espinales anteroventrales (AEAVs) exsangües, 4) hipotrofia de raicillas nerviosas en la hilera de los nervios IX, X y XI, 5) en algunos casos, hipotrofia de la superficie anterior de las pirámides e 6) hipotrofia de raíces anteriores en C5 - C6.Todos ellos recibieron transplante de epiplón a la superficie anterior, lateral y posterior de la medula oblongada y en 5 pacientes, un transplante adicional a nivel C5-C6. Resultados: La mejoría neurológica fue observada desde el primer día de la operación y fue mayor durante los primeros días o semanas de la cirugía que en los siguientes meses. Actualmente, 2 pacientes con 8 y 12 meses de evolución postoperatoria han mejorado en un 90% los síntomas de la forma bulbar de ELA. Conclusión: Estos resultados indican que la forma bulbar de ELA es originada por isquemia progresiva en el territorio intraparenquimatoso de las AEAVs y la forma espinal por isquemia en la arteria espinal anterior, pero a nivel C5 a T1. Eso explicaría porque su revascularización por medio del epiplón produjo mejoría neurológica.

Introduction: In August 2009 we performed surgery for the first time in a patient with amyotrophic lateral sclerosis (ALE). However, every published report about ALE mentions there is no cure for this condition. Objective: To prove that the progression of ALE may be stopped and the condition may be improved using an epiplon transplant. Material and Method: This report presents our experience with 13 ALE patients, affected by the bulbar and spinal forms of the disease. During the surgical procedures, we made the following findings: 1) anatomical variants in the V4 segment of vertebral arteries, 2) moderate or severe atherosclerosis in both V4 segments, 3) some circumflex blood vessels originating from the low-flow anterior and ventral spinal arteries, 4) hypothrophy of small nerve roots in the pathway of IX, X, and XI cranial nerves, 5) in some cases, hypothrophy of the anterior surface of the pyramids, and 6) hypothrophy of the anterior roots in C5 - C6. Every patient underwent an epiplon transplant upon the anterior, lateral, and posterior aspects of the medulla oblongata, and 5 patients underwent an additional transplant performed upon the C5-C6 territory. Results: Neurological improvement was seen from the first postoperative day, and it reached its maximum level during the first few days or weeks after surgery. Nowadays, 2 patients after eight and twelve months after surgery have had a 90% symptomatic improvement of the bulbar form of ALE. Conclusion: These results indicate that the bulbar form of ALE is caused by progressive ischemia in the parenchymal territory irrigated by the anterior and ventral spinal arteries, and that the spinal form is caused by ischemia in the anterior spinal artery, affecting the area between C5 and T1. This may explain why this revascularization procedure using epiplon tissue led to neurological improvement.

Relation between fractional anisotropy in the cerebral peduncles and changes in motor evoked potential after acute middle cerebral artery territory infarction / 国际脑血管病杂志

Objective To investigate the changing characteristics of the fractional anisotropy (FA) in cerebral peduncles and its relation with motor evoked potential (MEP) after acute cerebral infarction and to clear the clinical sigiificance of the low limit value of the FA in cerebral peduncles. Methods The low limit value of the FA in normal cerebral peduncles was determined based on mean - 1. 64 standard deviation. The patients with acute cerebral infarction (n = 58) were divided into MEP positive group and MEP negative group according to the absence and presence of MEP, in which the patients in the MEP positive group were redivided into the FA in cerebral peduncles ＜ the low limit value and≥ the low limit value groups according to the FA in cerebral peduncles on the affected sides. Results The low limit value of the FA in normal cerebral peduncles was 0. 36. There was significant difference in the FA in cerebral peduncles on the affected sides between the MEP negtive and MEP positive groups. The MEP negative group was the lowest (P=0. 000). The FA in cerebral peduncles on the affected sides in the positive group was significantly lower than that on the unaffected sides (P=0. 000), and the latency on the affected sides was longer than that on the normal sides (P=0. 000). The FA in cerebral peduncles on the affected sides was negatively correlated with the MEP latency (r=-0.332,P=0. 042). The MEP latency in the FA＜the low limit value group was significantly longer than that in the FA ≥ low limit value group (P=0. 002). There were no significant differences in the FA in cerebral peduncles on the normal sides and the MEP latency among an groups. The detection rate of the FA in cerebral peduncle＜0. 36 on the affected sides was the highest (50%). Conclusions In the evaluation of the prognosis of the patients, the changes of the FA in cerebral peduncles on the affected sides in patients with acute cerebral infarction had correlation,consistency, and complementarity with MEP.The detection rate of the low limit value of the FA in normal cerebral peduncles was the highest in the MEP negative patients. When the FA in cerebral peduncles was＜0.36 on the affected sides, particularly when MET was negative, it might predict that the prognosis was poor.

Effects of tetanic stimulation of peripheral nerve on intracranial direct electrical stimulation motor-evoked potentials in patients undergoing cerebral functional area operation / 中华麻醉学杂志

Objective To investigate the effects of tetanic stimulation of peripheral nerve on intracranial direct electrical stimulation motor-evoked potentials (MEP) in patients undergoing cerebral functional area operation. Methods Eight patients undergoing elective brain tumor resection under propofol-fentanyl anesthesia with partial neuromuscular blockade were enrolled in the study. Both conventional MEP (C-MEP) monitoring and posttetanic MEP (P-MEP) monitoring were performed throughout the operation for each patient, and the two groups of data were recorded. For one group, direct electrical stimulation with a train of five pulses was delivered to motor cortex and pyramidal tract, C-MEP was unilaterally recorded from the abductor pollicis brevis, and P-MEP was obtained 1 s after tetanic stimulation (frequency 50 Hz, intensity 50 mA, duration 5 s) to the ipsilateral tibial nerve.For the other group, direct electrical stimulation with a train of five pulses was delivered to motor cortex and pyramidal tract, C-MEP was unilaterally recorded from the tibialis anterior, and P-MEP was obtained 1 s after tetanic stimulation (frequency 50 Hz, intensity 50 mA and duration 5 s) to the contralateral tibial nerve. Randomized crossover method was used for C-MEP and P-MEP recording in each group, with an interval of 120 s. The adverse effects were observed. Results Amplitudes of P-MEP from the abductor pollicis brevis and tibialis anterior were significantly higher than those of C-MEP. Three patients had body movement during intraoperative cortex stimulation, while there was no awareness during operation and other electrical stimulation-related nervous system impairment and complications. Conclusion The application of tetanic stimulation of peripheral nerve before direct electrical stimulation can augnent the amplitudes of MEP from the abductor pollicis brevis and tibialis anterior in patients undergoing cerebral functional area operation.

Tracing rat nerve tracts with 7.0T Manganese-enhanced MRI / 中国医学影像技术

Objective To investigate the effect of manganese-enhanced MRI (MEMRI) at 7.0T for tracing nerve tracts in rat brain in vivo. Methods With brain stereotactic apparatus, 0.4 μl Mncl_2 with aqueous solution of 1 mol/L was injected into the right somatosensory cortex of 9 SD rats. MR scan was performed for tracing corticospinal tracts and other coherent nerve tracts pre-, and 24, 48, 72 h, 7 days post-injection with 7.0T micro-MRI system, respectively. Results Corticospinal tracts were showed in intact after Mn~(2+) administration from somatosensory cortex, thalamus, cerebral peduncle to pons at the time point of 24, 48, 72 h and 7 days, while the best tdisplaying was achieved at 24-48 h after Mn~(2+) administration. Simultaneously a small quantity of Mn~(2+) reached the opposite somatosensory cortex through the corpus callosum. Conclusion MEMRI for tracing rat nerve tracts can be showed clearly with 7.0T MRI. The location of manganese-enhanced corticospinal tracts in agreement with the rat brain atlas in stereotaxic is in agreement with that Paxinos' published. MEMRI can display the relationship between the two sides of hemisphere, and may play an important role in investigating the brain function and nerve plasticity after nerve injury in vivo.

Electrophysiological Evaluation of the Cortico-Diaphragmatic Pathway in Mechanical Ventilation of Patients with Chronic Obstructive Pulmonary Disease / 天津医药

Objective:To investigate the characteristics of phrenic nerve motor conduction (PNC)and motor evoked potentials of diaphragm elicited through magnetic stimulation (dMEP)in mechanical ventilation of patients with chronic obstructive pulmonary disease(COPD).Methods:PNC and dMEP were performed in 10 COPD patients with regular mechanical ventilation(the duration of mechanical ventilation≤7 d),10 COPD patients with prolonged mechanical ventilation(the duration of mechanical ventilation＞7 d),and 10 healthy subjects(control group).Results:There were significant differences in the latency and the common logarithm of the amplitude of PNC,cervical dMEP and cortical dMEP between three groups before and after weaning(P＜0.01 or P＜0.05).There were significant differences in central motor conduction time(CMCT)in three groups before and after weaning(P＜0.01).There was longer CMCT before weaning in two COPD groups than that of control group(P＜0.01).It showed no statistical difference in CMCT before weaning between two COPD groups(P＞0.05).There was a longer CMCT after weaning in prolonged mechanical ventilation than that of regular mechanical ventilation(P＜0.01).However,there was no statistical difference in CMCT after weaning between the regular mechanical ventilation group and the control group(P＞0.05).Conclusion:There were dysfunctions in the cortico-diaphragmatic pathway in COPD patients with mechanical ventilation,which may be aggravated by the prolonged mechanical ventilation.PNC and dMEP may help to define the cause of respiratory dysfunction in COPD patients with mechanical ventilation.

Diffusion tensor imaging in detection of Wallerian degeneration of pyramidal tract after cerebral infarction / 中华神经科杂志

Objective To investigate the evolution of diffusion indices in the pyramidal tract with Wallerian degeneration(WD)due to cerebral infarction using diffusion tensor imaging(DTI),and to study the relationship between early changes of diffusion indices and motor deficit.Methods Fifteen patients (13 males and 2 females)with acute cerebral infarction(within 7 days)were recruited from the Neurology Department from Mar 2006 to Jan 2007.A11 patients were assessed with DTI.National Institutes of Health Stroke Scale(NIHSS),Bathel Index(BI),modified Rankin Scale(mRS)and Motricity Index(MI)within 7 days from onset,and at the second week.DTI was performed with SIEMENS Trio 3.0 T MR scanner.The placement of region of interest(ROI),measurement of diffusion indices were performed by DTI Studio software.The mean diffusivity(MD),the fractional anisotropy(FA),the first eigenvalue (λ1),the second eigenvalue(λ2),and the third eigenvalue(λ3)were computed.Results At the second week.NIHSS was 6.93±3.39.BI 45.33±26.01,mRS 4.33±0.90.and MI 69.47± 60.71.At the second week from onset.MD of the pyramidal tract at the levels of the middle slice of pons and the superior slice of medulla oblongata showed no significant differences between both the two sides at second week from onset. Other ROI showed significant differences between both sides.MD.FA and λ1 of affected side were lower than the unaffected side.λ2 and λ3 of the affected side were higher than the unaffected side.Positive correlations were found between FA and BI(r=0.530,P=0.042),FA and MI(r=0.543,P=0.036)at the second week.Negative correlations were found between FA and NIHSS(r=-0.613,P=0.015)at the second week.Conclusions DTI can detect the changes in the pyramidal tract due to WD within 7-14 days after ischemic stroke.including a decrease of the fractional anisotropy.the first eigenvalue and increased the second and the third eigenvalues.The fractional anisotropy of the second week from onset is related to the outcome of the motor function.

A Case of Osmotic Myelinolysis Confined to Central Pons and Pyramidal Tracts

Osmotic myelinolysis is a distinctive clinical syndrome with a characteristic "bat wing" MRI lesion in the central pons, which is also called central pontine myelinolysis (CPM). However, demyelinating lesions are not only limited to the central pons, but also may be involved in the extrapontine regions including the basal ganglia, thalamus, and cerebellum (extrapontine myelinolysis; EPM). We report an atypical case of osmotic myelinolysis confined to the pyramidal tract from the precentral gyrus to the central pons via the corona radiata and internal capsule in a MR image.

Quantitative study of corticospinal tract in patients with multiple sclerosis using diffusion tensor tractography / 中华放射学杂志

0.05). The average FA of the corticospinal tracts (0.472?0.037) of RRMS group was significantly lower than that of normal control group (0.497?0.028) (P0.05). The average FA of the corticospinal tracts correlated with EDSS (r=-0.193, P0.05). Conclusion The average FA of the corticospinal tracts of RRMS patients is significantly abnormal and this index may be suitable in evaluating the functional status of the cerebral type of RRMS patients.

Central Hypoventilation Syndrome during Awake and Sleep

Central hypoventilation syndrome (CHS) can be caused by any lesions to the medullary respiratory centers, cerebral cortex, corticospinal pathways, and their connections. We report 5 patients with central hypoventilation syndrome and analyzed 26 patients who experienced central hypoventilation syndrome during sleep and waking states. We compared initial clinical symptoms and signs, maximal neurologic deficits, brain MRI and pathologic findings, and associated autonomic dysfunctions. The patients with respiratory failure during waking states showed quadriplegia, a rapidly progressing respiratory failure. The patients who had automatic respiratory failure showed mild hemiparesis, bulbar dysfunction, dysautonomia, and subacute to chronic recurrent respiratory failures. These results support the concept of two separate respiratory systems: a voluntary system and an automatic system. The respiratory management of these patients with central hypoventilation syndrome should be considered critical to their survival.

Preliminary assessment of amyotrophic lateral sclerosis by using MRI and MR diffusion tensor imaging / 中华放射学杂志

Objective To describe the characteristic MR findings in the brain in patients with amyotrophic lateral sclerosis (ALS), and to assess the diagnostic value of conventional MR imaging and fractional anisotropy (FA) of diffusion tensor imaging (DTI). Methods Conventional MR imaging was performed in 14 clinically proved ALS patients and 12 age-matched normal controls. Contrast enhanced MR images were acquired in 2 patients. Axial and coronal DTI scans were performed in 10 patients and 12 normal controls with SE-EPI sequence. The b value was 1 000 s/mm 2, the number of diffusion sensitive gradient direction was 25. For quantitative assessment of the corticospinal tract (CST), FA value of bilateral CST was measured at the level of posterior limb (PL) of the internal capsule (IC) and the cerebral peduncle of the midbrain, respectively, and statistical analysis was performed. Results Focal slight low signal intensity on T 1WI and high signal intensity (hyperintense to gray matter) on T 2WI was demonstrated in 6 ALS cases (42.9%) in bilateral PL of the IC, and the high signal was longitudinally continuous from the PL to the cerebral peduncle on T 2WI coronal plane, corresponding to the course of CST. In another 8 ALS cases (57.1%), the focal slight low signal intensity on T 1WI and slight high signal intensity (isointense to gray matter) on T 2WI was revealed in bilateral PL of the IC. No abnormal contrast enhancement was detected in the 2 cases. In control group, the focal slight low signal intensity on T 1WI and slight high signal intensity (isointense to gray matter) on T 2WI was demonstrated in all 12 subjects in bilateral PL of the IC. FA values of the patient group were significantly lower than that of the control group at the level of the PL of the IC (F=7.38, P