Prenatal diagnosis of fetal diencephalic-mesencephalic junction dysplasia: a case report / 中华围产医学杂志

We report a case of fetal diencephalic-mesencephalic junction dysplasia (DMJD) diagnosed prenatally. Prenatal ultrasound at 24 gestational weeks showed that the fetus was small, about the size at 22 weeks' gestation, with short biparietal diameter and enhanced echo at the anterior border of thalamus. Fetal MRI showed short T2 signal shadow in the left choroid plexus, and hemorrhage and midbrain dysplasia were suspected. A pathogenic homozygous mutation variant in protocadherins 12 gene (c.1558C>T) was found in this fetus by whole exome sequencing and both parents carried the same heterozygous variation revealed by Sanger sequencing. All of the above information lead to the diagnosis of fetal DMJD, and the pregnancy was terminated after genetic counseling.

Spinal cord atrophy-like changes in adolescent Chiari malformation type I patients: a neglected imaging change / 中华骨科杂志

Abstract:To compare the morphology of spinal cord between healthy adolescents with adolescent Chiari malformation type I (CMI) patients and investigate the impact of syringomyelia on the morphology of spinal cord in CMI patients.Methods:The clinical and radiological data of 292 CMI patients diagnosed by our center between June 2012 and March 2019 were retrospectively reviewed. Among them 15 CMI patients without syringomyelia were recruited in the CM group. Among the remaining 277 CMI patients, 274 patients had syringomyelia below the C 3-4 intervertebral disc. According to the principle of best matching, CMI patients with syringomyelia were selected with the closest age to the CM group (±18 months), and 30 CMI patients with syringomyelia were included in the CMS group according to a ratio of 1∶2. Thirty healthy adolescents were enrolled as the control group (NC group) in the same way. The anteroposterior diameters of spinal cord at C 2 (DSCO-C 2), spinal canal at C 2 (DSCA-C 2), midbrain-pontine junction (DPJ), the distance between the tip of cerebellar tonsils and the foramen magnum (AB) and the maximal diameter of the syrinx (D-syrinx) were measured on MRI. All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. One-way ANOVA and SNK- q test were used to compare the above radiographic parameters and age between CM, CMS and NC group. The distribution of genders was compared between the three groups using Chi-square tests. Pearson correlation analysis were conducted to demonstrate the relationship between radiographic parameters in CM and CMS group. Results:ICC ranged between 0.91 and 0.95 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. Patients in CM and CMS groups showed similar DSCO-C 2 values ( P=0.254), both of which were significantly lower than that in NC group ( P<0.001). DSCA-C 2 in CMS group was significantly larger than that in CM ( P=0.003) and NC ( P<0.001) groups, while no significant difference was found between the CM and NC groups ( P=0.216). Moreover, DPJ in CMS group was significantly lower than that in CM group ( P<0.001) and NC group ( P<0.001). There was no significant difference in AB between CM and CMS groups ( P=0.948). DSCO-C 2 was significantly positively correlated with DSCA-C 2 in CMS group ( r=0.906, P<0.001), while AB, D-syrinx, DSCO-syrinx, DSCA-syrinx and DPJ were not significantly correlated with DSCA-C 2. There were significant correlations observed between DPJ and other radiographic parameters in the CMS group (all P>0.05). Significant positive correlation between DSCO-C 2 and DPJ was observed in CM group ( r=0.703, P=0.005). There was no significant correlation between DSCO-C 2 and DSCA-C 2 and DPJ in NC group (all P>0.05). Conclusion:CMI adolescents have significant atrophic change of cervical spinal cord and midbrain-pontine junction compared with healthy adolescents, regardless of the existence of syrinx. Moreover, syrinx in CMI patients indicated more obvious atrophic change of midbrain-pontine junction and dilated spinal canal compared with isolated CMI patients.

A case of jerky seesaw nystagmus caused by mesencephalic and thalamic infarcts / 中华神经科杂志

Seesaw nystagmus is a rare spontaneous ocular movement,which refers to mixed torsional-vertical nystagmus with conjugate torsional components and the vertical components in the opposite directions.It is divided into the pendular and jerky types,according to the movement features.Seesaw nystagmus has not been reported in China.We herein report a case of jerky seesaw nystagmus caused by mesencephalic and thalamic infarcts,in order to improve awareness about the ocular sign.

Effect of light intensity on expression of gonadotropin-releasing hormone in mesencephalon and diencephalon of Jinghong-1 hens / 解剖学报

Objective To explore the nervous regulating mechanism in the effect of light intensity on reproductive performance. Methods Sixty healthy Jinghong-1 hens aged 100 days were randomly divided into four groups of 15 hens and applied a light of 30 lx, 20 lx, 10 lx, and 1 lx, respectively, for 7 days. The distribution and expression of gonadotropin-releasing hormone (GnKH) in mesencephalon and diencephalon were detected using immunohistochemical and image analysis methods. Results GnKH immunoreactive positive neurons were mainly observed in stratum griseum centrale (SCC) of tectum, nucleus isthmi pars magnocellularis (Imc) , nucleus isthmi pars parvocellularis (Ipc) , nucleus subpre-tectalis/interstito-pretectalis-subpretectalis (SP/IPS), nucleus of basal optic root (nbor), and nucleus semilunaris (Slu) of mesencephalon. The mean gray values of positive neurons in 30 lx and 20 lx groups were significantly higher than those of 10 lx and 1 lx groups (P0. 05) between 30 lx and 20 lx groups, but they were significant higher than those of 10 lx and 1 lx (P<0. 05) in PHN, PVN, and GLv. The percent cell area in Hot was the largest in 30 lx group, having no significant difference comparing 20 Ix, being significant higher than those of 10 lx and I Ix (P<0. 05). Those in 30 lx and 20 lx groups were significant higher than 10 lx and 1 lx (P<0.05) in PHN and PVN. Conclusion Light intensity affect the expression of GnRH in hen mesencephalon and diencephalon obviously in Jinghong-1 hens aged 100 days, regulating their reproductive function. The tectofugal pathway of the optical pathways, including collateral circulation of nucleus isthmi, plays an important role in the course of regulation.

Value of quantitative midbrain measurement in differentiating progressive supranuclear palsy from Parkinson's disease and multiple system atrophy / 中华神经科杂志

Objective To evaluate the effectiveness of quantitative midbrain measurements in differentiating progressive supranuclear palsy (PSP) from multiple system atrophy (MSA) and Parkinson's disease (PD).Methods Quantitative midbrain measurements,including midbrain width (MW),midbrain to pons ratio (M/P) and magnetic resonance parkinsonism index (MRPI),were performed in patients with parkinsonism who were diagnosed in the Movement Disorder Clinic of Peking Union Medical College Hospital during the period of January to September 2017.A cross-sectional study was conducted in the series to evaluate the effectiveness of these quantitative measurements.Results Ten PSP-RS,15 MSA-P and 49 PD patients were included in this study.The values of MW,M/P and MRPI in PSP-RS patients were (8.21 ± 1.30) mm,0.49 ±0.06 and 15.26 ±4.53,respectively,with statistically significant difference compared toMSA-P ((10.24±0.77) mm,0.65 ±0.09,7.75 ±2.71) and PD patients ((10.53 ±0.93) mm,0.62±0.06,9.86 ±2.46;F=24.27,18.37,21.47,all P＜0.01).After adjusting for age,disease course and gender,analysis of covariance suggested significant correlation between the quantitative midbrain measurements and diagnosis of these diseases.Receiver operating characteristic curve indicated that MW ≤9.4 mm,M/P≤0.57 and MRPI ＞ 10.77 showed ideal sensitivity and specificity (90.0％ and 92.1％,80.0％ and 93.7％,100.0％ and 82.5％) in differentiating PSP-RS from MSA-P and PD.Conclusion Quantitative measurement of midbrain atrophy is useful in differentiating PSP-RS from MSA-P and PD.

Retracción palpebral de causa neurológica: presentación de tres pacientes, interpretación patogénica / Eyelid retraction of neurologic origin: report of three cases

Eyelid retraction, has received limited attention and it has passively been interpreted as the result of an overactive levator palpebrae superioris muscle secondary to midbrain injury. However, eyelid retractions can occur in other neurological diseases, not directly related with the midbrain. We report three patients who developed eyelid retraction. One patient had a bilateral eyelid retraction, related with Creutzfeldt-Jakob disease (CJD). Another patient had a unilateral right eyelid retraction associated with a thalamic-mesencephalic infarct. The third patient had a bilateral pontine infarction on magnetic resonance imaging. In the patient with CJD, eyelid retraction did not subside. Among patients with infarctions, the retraction persisted after focal symptoms had subsided, showing an evolution that was apparently independent of the basic process. The analysis of these patients allows us to conclude that the pathogenesis of eyelid retraction includes supranuclear mechanisms in both the development and maintenance of the phenomenon. Unilateral or bilateral eyelid retraction does not alter the normal function of eyelid, which ever had normal close eye blink. In these reported cases, a hyperactivity of levator palpebrae superioris muscle was clinically ruled out.

Nonaneurysmal perimesencephalic subarachnoid hemorrhage: clinical and imaging features / 国际脑血管病杂志

ObjectiveTo investigate the clinical and imaging features of nonaneurysmal perimesencephalic subarachnoid hemorrhage (NAPSAH).MethodsThe patients with NAPSAH were retrospectively enrolled, and their clinical and imaging features were collected and analyzed.Results In 489 patients with subarachnoid hemorrhage treated over the same period, a total of 23 (4.7%) with NAPSAH were diagnosed with cranial CT and CT angiography.Subarachnoid blood centered in the prepontine cistern in 4 patients (17.39%), interpeduncular cistern in 8 (34.78%), ring pool in 7 (30.43%), and quadrigeminal cistern in 4 (17.39%).Two patients were transferred to other hospital for treatment, and the remaining 21 patients had a good outcome without complications and neurological deficits.Among them, the modified Rankin scale scores in 17 patient were 0, and 4 were 1.Their cardinal symptoms were forgetfulness, irritability, anxiety, and other subjective symptoms.They were improved after receiving psychological counseling and anti-anxiety treatment.ConclusionsThe incidence of NAPSAH is low.The clinical symptoms in patients with NAPSAH are mild, the outcomes are better without obvious complications.

Effects of Duliang capsule on the expression of calcitonin gene-related peptide and cholecystokinin in the midbrain of a rat migraine model / 重庆医学

Objective To study the effects of Duliang capsule on the expression of calcitonin gene‐related peptide(CGRP) and cholecystokinin(CCK) in the midbrain of a rat migraine model ,and explored treatment effects and mechanisms of Duliang cap‐sule on rats with migraine .Methods A total of 24 rats were randomly divided into four groups :normal control groups(A) ,migraine model groups(B) ,Duliang capsule control groups(C) and Duliang capsule treatment groups(D) .C and D were intragastrically per‐fused with Duliang capsule(0 .5 g · kg -1 · d-1 ) .After 7 days ,nitroglycerin was subcutaneously injected into the buttocks of the B and D to induce migraine .Two hours after nitroglycerin injection ,the midbrain were isolated CGRP and CCK expression in midbrain were determined using SYBR Green I real‐time quantitative PCR .Results CGRP mRNA expression was significantly lower in mid‐brains of rats in the Duliang capsule treatment groups compared with migraine model groups(P<0 .05) .CCK mRNA expression was significantly lower in midbrains of rats in the Duliang capsule control groups compared with normal control groups(P<0 .05) . Conclusion Duliang capsule can exhibit the expression of CGRP and CCK in the midbrain of the migraine rats .weaken the CGRP and CCK‐induced inhibition of the analgesic effects of opioid peptides .

Clinical and imaging features of precheron artery infarction / 国际脑血管病杂志

Objective To investigate the imaging and clinical features of Percheron artery infarction.Methods The clinical manifestations,imaging data,treatment,and prognosis of 10 patients with Percheron artery infarction were analyzed.Results All 10 patients had acute onset and had different degrees of consciousness disorders,including vertical gaze palsy (n =3),language disorder (n =3),limb weakness (n =6),and memory impairment (n =4).MRI showed bilateral thalamus infarction.Midbrain V-sign in one patient was observed.Cerebral angiography revealed that one patient had Percheron artery infarction.All 10 patients were improved and discharged,but left with varying degrees of sequelae.Conclusions Percheron artery infarction is rare in clinical practice.The diagnosis is mainly based on clinical manifestations and imaging examinations.Cerebral angiography can diagnose the disease.

Non-aneurysmal perimesencephalic subarachnoid hemorrhage / 国际脑血管病杂志

As a special type of subarachnoid hemorrhage,the prognosis of non-aneurysmal perimesencephalic subarachnoid hemorrhage (NAPSAH) is good.In recent years,with the development and popularization of neuroimaging technology,people have gradually deepened the awareness of the disease.This article reviews the progress in the diagnosis and treatment of NAPSAH.

Functional Neuroanatomy of Brain Stem

The brain stem consists of medulla oblongta, pons and midbrain. It is sited in posterior cranial fossa. It contains numerous intrinsic neuron cell bodies and their processes, some of which are the brain stem homologues of spinal neuronal groups. These include the sites of termination and cells of origin of axons that enter or leave the brain stem through the cranial nerves. Cranial nerves provide sensory, motor and autonomic innervations of structures that are mostly in the head and neck. The reticular formation is an extensive network of neurons that extends throughout the length of brain stem and is continuous rostrally to diencephalon and caudally to its spinal counterpart. Clinically, damage to the brain stem is often devastating and life threatening. This is because it is a structurally and functionally compact region. Therefore, it is important to build basic knowledge about neuroanatomy of brain stem.

Microanatomical study of the endoscopy-assisted extreme lateral supracerebellar infratentorial keyhole approach to the dorsolateral mesencephalon region / 中华显微外科杂志

Objective To explore the microanatomy technique of the endoscopy-assisted extreme lateral supracerebellar infratentorial keyhole approach and clinical significance.Methods The extreme lateral supracerebellar infratentorial keyhole approach was imitated in 10 vascular perfusion adult cadaveric heads fixed in formalin,the dorsolateral mesencephalon region was dissected and observed by microscopy and neuroendoscopy from December 2012 to March 2013.Results The extreme lateral supracerebellar infratentorial keyhole approach could be fully exposed the ambient cisterns,the posteromedial tentorial incisura,the dorsolateral mesencephalon region,the trochlear nerve,the root of the trigeminal nerve,the superior cerebellar artery,the posterior cerebral artery and other important anatomical structures.The temporal lobe and parahippocampal gyrus could be exposed when the tentorial was incised.The application of neuroendoscopy could clearly reveal the trigeminal nerve and surrounding structures.Conclusion The extreme lateral supracerebellar infratentorial keyhole approach is suitable for the surgical resection in dorsolateral mesencephalon region.The application of neuroendoscopy can make up for the lack of the trigeminal nerve observed under microscopy and expand the operative field.

Diagnositic value of trascranial sonography in detection of Parkinson disease / 中华医学超声杂志（电子版）

Objective To evaluate the clinical value of trascranial sonography (TCS) in measuring hyperechogenic substantia nigra ( SN) area and area ratio of SN to midbrain ( S/M) for the diagnosis of Parkinson′s disease( PD).Methods A total of 109 PD patients ( PD group) and 115 normal controls (control group) underwent TCS.The area of midbrain and SN and the area ratio of S/M were measured and compared between PD group and control group .Statistical analysis of the two parameters in predicting PD was performed with receive operating characteristic ( ROC) curves.The sensitivity and specificity of each parameter and their combination were calculated .Results The hyperechogenic SN area and S/M were (0.34 ±0.27)cm 2 and (12.15 ±4.57)%in PD group,whereas (0.14 ±0.08)cm 2 and (6.37 ±3.30)%in control group respectively .The difference between the two groups was statistically significant (t=82.68, 100.83,both P7.52%,the sensitivity was 80.4%and the specificity was 78.6%.Conclusions TCS is a fast, convenient,effective and useful tool for screening PD .The combination of the SN area and S/M provided the best diagnostic parameters.

Vertical One-and-a-Half Syndrome With Bilateral Pseudo-Abducens Palsy From Unilateral Thalamo-Mesencephalic Infarction

No abstract available.

Hemiparkinsonism associated with mesencephalic cavernoma: case report and review of literature

Parkinsonism is a movement disorder characterized by resting tremor, slow and decreased movements (hypokinesia and akinesia), rigidity, postural instability, problems with gait, and coordination. Parkinson?s disease (PD) is the most common cause of parkinsonism and its prevalence is estimated to range from 0.1 per cent to 0.3 per cent in the general population and from 1 per cent to 2 per cent in persons 65 years of age or older. Although the majority of cases of PD are describe to be sporadic, many identifiable etiologies have been included as possible causes of parkinsonism, such as genetic disorders, cerebrovascular events and intoxication. However, mesencephalic cavernoma is an extremely rare condition associated to hemiparkinsonism. In the present report, we describe the case of a Brazilian woman that evolved symptoms of hemiparkinsonism and presented a ventral mesencephalic cavernoma on radiological investigation.

O parkinsonismo corresponde a um transtorno do movimento caracterizado por tremores de repouso, redução e lentidão dos movimentos (hipocinesia e acinesia), rigidez, instabilidade postural e problemas relacionados a marcha e coordenação. A doença de Parkinson (DP) é a causa mais comum de parkinsonismo e sua prevalência é estimada em torno de 0,1 por cento a 0,3 por cento em relação à população geral e 1 por cento a 2 por cento em pessoas acima de 65 anos de idade. Embora a maioria dos casos de DP tenha sido descrita como casos esporádicos, muitas etiologias identificáveis foram incluídas como possíveis causas de parkinsonismo, tais como alterações genéticas, eventos cerebrovasculares e intoxicações. No entanto, o cavernoma mesencefálico é uma causa de hemiparkinsonismo extremamente rara. No presente relato, descreve-se o caso de uma mulher brasileira que apresentou sintomas de hemiparkinsonismo relacionados à presença de um cavernoma mesencefálico ventral na investigação radiológica.

Effect of dexmedetomidine on norepinephrine release in midbrain periaqueductal gray in a rat model of incisional pain / 中华麻醉学杂志

Objective To investigate the effect of dexmedetomidine on norepinephrine(NE)release in midbrain periaqueductal gray(PAG)in a rat model of incisional pain.Methods Twenty-four male Wistar rats in which microdialvsis catheter was successfully placed in the ventrolateral region of PAG without complications were randomly divided into 4 groups(n=6 each):group control(group C);group incisional pain(group IP);group dexmetomidine(group D)and group dexmedetomidine+yohimbine(group DY).Incisional pain was induced by an incision made into the plantar surface of left hindpaw in IP,D,DY groups.Dexmedetomidine 30 μg/kg and dexmedetomidine 30 μg/kg+yohimbine 0.5 mg/kg were given intraperitoneally at 15 min before plantar incision in group D and group DY respectively.Mechanical paw withdrawal threshold(MWT)to von Frey filament stimulation was measured at 30 min before(baseline)and 1,2,3,4 h after operation in C,IP,D groups,and at 30 min before(baseline),and 1 h after operation in group DY.Dialysate samples were collected at 30 min before(baseline)and at evcry 30 min after operation for 4 h via cerebral microdialysis catheter for determination of the NE concentration in C,IP,D groups,and at 30 min before(baseline),30,60 min after operation in group DY.Results Incisional pain significantly decreased MWT and increased the NE concentration in dialysate in group IP.Dexmedetomidine premedication significantly inhibited mechanical hyperalgesia and attenuated incisional pain-induced increase in the NE concentration in dialysate in group D.Yohimbine counteracted effects of dexmedetomidine.Conclusion Dexmedetomidine has analgesic effect though inhibition of NE release from PAG.

Expression changes of microRNA array in mesencephalon of rats with traumatic coma / 中华创伤杂志

Objective To set up a rat model with acute traumatic coma and identify the variation of microRNA in mesencephalon. Methods After rats were injured moderately by central fluid percussion system, tissues of the mesencephalon were removed one hour after injury. RNA of brain tissue of the mesencephalon was isolated for microRNA array by using the exiqon microarray system. The data were analyzed statistically by Genepix Pro 6.0 after hybridization results were scanned and fluorescence intensity standardized. Resets Hybridization results showed 33 microRNAs with up-regulated expressions but 38 microRNAs with down-regulated activity. Conclusion Expression of microRNA array shows marked changes in the tissues of the mesencephalon in rats with traumatic coma, as may be injury mechanism of traumatic coma and also a way of neurobiological protection of coma.

The CK1 gene family: expression patterning in zebrafish development

Protein kinase CK1 is a ser/thr protein kinase family which has been identified in the cytosol cell fraction, associated with membranes as well as in the nucleus. Several isoforms of this gene family have been described in various organisms: CK1 , CK1á, CK1δ, CK1å and CK1γ. Over the last decade, several members of this family have been involved in development processes related to wnt and sonic hedgehog signalling pathways. However, there is no detailed temporal information on the CK1 family in embryonic stages, even though orthologous genes have been described in several different vertebrate species. In this study, we describe for the first time the cloning and detailed expression pattern of five CK1 zebrafish genes. Sequence analysis revealed that zebrafish CK1 proteins are highly homologous to other vertebrate orthologues. Zebrafish CK1 genes are expressed throughout development in common and different territories. All the genes studied in development show maternal and zygotic expression with the exception of CK1å. This last gene presents only a zygotic component of expression. In early stages of development CK1 genes are ubiquitously expressed with the exception of CK1å. In later stages the five CK1 genes are expressed in the brain but not in the same way. This observation probably implicates the CK1 family genes in different and also in redundant functions. This is the first time that a detailed comparison of the expression of CK1 family genes is directly assessed in a vertebrate system throughout development.

A PRELIMINARY RESEARCH IN CULTIVATION AND DIFFERENTIATION OF CORTICAL AND MESENCEPHALIC NEURAL STEM CELLS IN VITRO / 解剖学报

Objective To explore the differences of cultivation and differentiation between cortical neural stem cells(C-NSCs) and mesencephalic neural stem cells(M-NSCs). Methods We dissociated embryonic cortex and ventral mesencephalon respectively,and single cell suspensions were achieved by trypsin digestion and mechanical dissociation in sterile condition.These cells were planted into DMEM/F12 culture medium with B27,bFCF,and then were passaged by mechanical methods and induced to differentiation in DMEM/F12 medium with 10%FBS(without growth factors).For examining the features of C-NSCs and M-NSCs,reverse phasecontrast microscopy,immunocytochemical techniques were applied to detect nestin,NSE,GFAP antigens and the specific antigen(TH) of neurons differentiated from the two kinds of NSCs.We observed and compared the difference of the two kinds of NSCs. Results The NSCs existed both in cortex and ventral mesencephalon of embryonic mice,which expressed nestin and possessed the ability of self-renewing and multipotent, but the density of NSCs in cortex was higher than that in ventral mesencephalon.There were some TH positive cells differentiated from M-NSCs but none came from C-NSCs.Conclusion The proliferative activity of C-NSCs is higher than that of M-NSCs,but more number M-NSCs differentiated into dopaminergic neurons in serum medium.

Transplantation of mesencephalon progenitor cells in treatment of Parkinsonian rats / 第二军医大学学报

Objective:To explore the feasibility of mesencephalon progenitor cells as dopamine neuron's graft in treatment of Parkinson disease(PD) in rats. Methods: Animal model of PD were established in SD rats by 6 hydroxydopamine. Mesencephalon progenitor cells were obtained from 13 day old fetal rats. After in vitro proliferation and differentiation, the cells were planted into PD rat striatum. Results: Progenitor cells were expanded in an undifferentiated state with endothelia growth factor and basic fibroblast growth factor as mitogens. IL 1 and striatal culture media further increased the number of TH immunoreactive cells. Transplantation of these neurons relieved the rotation symptom of PD rats. Conclusion: Transplantation of mesencephalon progenitor cells has certain effects on PD rats.