Metameric Distribution Research Articles

The Trigeminal Herpes Zoster and the Hutchinson Sign Presentation of 5 Cases and Literature Review

Herpes Zoster is an acute disease, with unilateral metameric distribution, which is characterized by the development of vesicles grouped into an inflammatory, erythematous plaque, located on the path of a nerve.

The cerebrofacial metameric syndromes: An embryological review and proposal of a novel classification scheme.

The cerebrofacial metameric syndromes are a group of congenital syndromes that result in vascular malformations throughout specific anatomical distributions of the brain, cranium and face. Multiple reports of patients with high-flow or low-flow vascular malformations following a metameric distribution have supported this idea. There has been much advancement in understanding of segmental organization and cell migration since the concept of metameric vascular syndromes was first proposed. We aim to give an updated review of these embryological considerations and then propose a more detailed classification system for these syndromes, predominately incorporating the contribution of neural crest cells and somitomeres to the pharyngeal arches.

Impact of the cystic neural tube defects on fetal motor function in prenatal myelomeningocele repairs: Aretrospective cohort study.

To determine the impact of the lesion type (cystic [myelomeningocele] or flat [myeloschisis]) on the fetal motor function (MF) in cases candidates for prenatal open neural tube defect (ONTD) repair. Retrospective cohort study of patients with ONTD who underwent prenatal repair at a single institution between 2011 and 2019. The lesion type and the measurements of the length and width of the lesions to calculate the surface of the ellipsoid lesion were performed using MR scans. Prenatal MF of the lower extremities was evaluated by ultrasound following a metameric distribution at the time of referral. Intact MF was defined as the observation of plantar flexion of theankle. Logistic regression was performed to determine the predictive value of the type of lesion for having an intact MF at the time of referral. 103 patients were included at 22.9 (19-25.4) weeks; 65% had cystic and 35% had flat lesions. At the time of referral, there was a higher proportion of cases with an intact MF in the presence of flat lesions (34/36; 94.4%) as compared to cystic lesion (48/67; 71.6%, p<0.01). When adjusting for gestational age and anatomical level of the lesion, flat ONTD were 3.1 times more likely to be associated by intact motor function (CI%95 [2.1-4.6], p<0.01) at the time of referral. Cystic ONTD are more likely to be associated with impaired MF at mid-gestation in candidates for prenatal ONTD repair.

Prenatal predictors of motor function in children with open spina bifida: a retrospective cohort study.

To identify predictors for intact motor function (MF) at birth and at 12months of life in babies with prenatally versus postnatally repaired open spina bifida (OSB). Retrospective cohort study. Texas Children's Hospital, 2011-2018. Patients who underwent either prenatal or postnatal OSB repair. Prenatal MF of the lower extremities was evaluated by ultrasound following a metameric distribution at the time of diagnosis (US1), 6weeks postoperatively (or 6weeks after initial evaluation in postnatally repaired cases) (US2) and at the last ultrasound before delivery (US3). At birth and at 12months, MF was assessed clinically. Intact MF (S1) was defined as the observation of plantar flexion of the ankle. Results from logistic regression analysis are expressed as odds ratios (95% confidence intervals, P values). A total of 127 patients were included: 93 with prenatal repair (51 fetoscopic; 42 open hysterotomy repair) and 34 with postnatal repair. In the prenatal repair group, predictors for intact MF at birth and at 12months included: absence of clubfeet (OR11.3, 95%CI 3.2-39.1, P<0.01; OR10.8 95%CI 2.4-47.6, P<0.01); intact MF at US1 (OR19.7, 95%CI 5.0-76.9, P<0.01; OR8.7, 95%CI 2.0-38.7, P<0.01); intact MF at US2 (OR22, 95%CI 6.5-74.2, P<0.01; OR13.5, 95%3.0-61.4, P<0.01); intact MF at US3 (OR13.7, 95%CI 3.4-55.9, P<0.01; OR12.6, 95%CI 2.5-64.3, P<0.01); and having a flat lesion (OR11.2, 95%CI 2.4-51.1, P<0.01; OR4.1, 95%CI 1.1-16.5, P=0.04). In the postnatal repair group, the only predictor of intact MF at 12months was having intact MF at birth (OR15.2, 95%CI 2.0-113.3, P=0.03). The detection of intact MF inutero from mid-gestation to delivery predicts intact MF at birth and at 12months in babies who undergo prenatal OSB repair. Detection of intact motor function inutero predicts intact motor function at birth and at 1year in fetuses who undergo prenatal OSB repair.

Cobbs syndrome: A case of spinal arteriovenous malformation treated with endovascular embolization

Abstract Cobb syndrome is a rare illness that combines vascular skin nevus and spinal vascular malformation at the same metamere. We report the case of a 34-year old man that presented a light low back pain, a slowly progressive paraparesis and abnormal urinary sphincter functioning. MR imaging of the thoracic spine depicted an intramedular arteriovenous malformation. Spinal angiography confirmed these findings. The arteriovenous malformation also affected the chestwall bones and muscles with the same metameric distribution as the intramedular lesion. Due to these findings Cobb syndrome was suspected and in a second physical examination a skin angioma was noted on the patient's back. He was treated with corticosteroids and endovascular coiling and his symptoms rapidly improved. Although pathophysiology of this syndrome is poorly understood and the correct management remains unknown, endovascular treatment has become the first choice for spinal arteriovenous malformations. We believe that combined treatment with corticosteroids and endovascular embolization is an effective procedure to alleviate symptoms and to minimize morbidity.

Cerebrofacial Venous Metameric Syndrome 2 Plus 3: Facial and Cerebral Manifestations

A unique case of Cerebrofacial Venous Metameric Syndrome (CVMS) in a 25-year-old women is described with venous malformations involving simultaneously the brain and the face. This case represents CVMS 2 plus 3 according to the classification described by Lasjaunias et Al. The metameric distribution of the cerebrofacial venous syndromes is well illustrated in this case. The extracranial and intracranial involvement is described and the appearance on MRI imaging is detailed. In our opinion this case demonstrates that cerebrofacial venous metameric syndromes include a wide spectrum of possible phenotypes including Sturge Weber syndrome and cases such as we describe here.

Vitíligo segmentario asociado a traumatismo quirúrgico

Resumen —El vitiligo segmentario es una dermatosis infrecuente caracterizada por una distribucion metamerica. Se presenta el caso de un varon de 20 anos de edad con un vitiligo segmentario, que se inicio tras una intervencion quirurgica en las proximidades del area metamerica afectada.

Acute traumatic central cord syndrome: magnetic resonance imaging and clinical observations.

Acute traumatic central cord syndrome has been classically thought to be caused by a hemorrhage that primarily affects the central part of the spinal cord and that destroys the axons of the inner part of the corticospinal tract devoted to the motor control of the hands. Some authors, however, have proposed that its pathogenesis is based on the destruction of the motor neurons supplying the muscles of the hand. To test the validity of these two theories, the authors retrospectively studied the magnetic resonance (MR) images obtained in 18 cases of acute traumatic central cord syndrome (ATCCS) to assess the presence of intramedullary blood and to define the distribution of the abnormal signal intensities in the cervical spinal cord. The authors used the American Spinal Injury Association (ASIA) motor scale to assess upper- and lower-limb deficits and to evaluate its metameric distribution. The abnormal intramedullary signal was then compared with the distribution of the motor deficit. All MR imaging sessions performed in the acute stage revealed a hyperintense signal on T2-weighted sequences without any signal change suggesting the presence of intramedullary blood. The localization of this signal was distributed predominantly from the C3-4 to the C5-6 disc levels. The mean ASIA motor score was 74.3 of 100, with an unequal representation between the upper and lower limbs (32 of 50 compared with 42.3 of 50, respectively). The metameric distribution of the deficit was also unequal, with a major deficit in hand function (C8-T1) compared with the more proximal cord segments (5.2 of 10 compared with 7.8 of 10, respectively). This demonstrates the absence of any correlation between the hyperintense signal and the motor deficit distribution. Acute traumatic central cord syndrome cannot be explained by the injury to the gray matter at the level of motor neurons supplying the hand muscles. In agreement with recently published data, the results of this series confirm the absence of intramedullary hemorrhage and corroborate the hypothesis that ATCCS may be explained by the impairment of the corticospinal tract, which can be affected globally.

Trifocal Monomyelomeric Spinal Cord Arteriovenous Fistulae in a Seven-Year-Old Boy

We describe a rare case of multiple arteriovenous fistulae of the spinal cord (SCAVF) in the same myelomer in a five-year-old boy. This case report consists of a trifocal SCAVF at the Th12 myelomeric level without communication between the three different fistulae. Two AVF were located posteriorly, bilateraly, in the spinal cord, fed by left and right posterior radiculopial arteries and one anteriorly in the anterior spinal axis. The venous drainage was independent for each lesion. The patient presents associated lesions characterized by cutaneous stain and inferior limb asymmetry. A metameric distribution is the explanation for the multiplicity of these lesions in a syndromic association related to Cobb syndrome. The patient was treated by transarterial embolization using glue with occlusion of the three different fistulae. The patient achieved a good improvement in neurological status.

Multifocal and Metameric Spinal Cord Arteriovenous Malformations

We describe 19 (16.0%) multiple vascular malformations (AVMs) in 119 spinal cord arteriovenous malformations (SCAVMs). The associated lesions were eight vertebral vascular malformations, two cutaneous, four limbs, four radicular AVMs, three bifocal SCAVMs; one patient had a bifocal cord lesion associated with vertebral and limb localisations. Various syndromic associations were seen: nine Cobb, two Klippel-Trenaunay-Weber, one Parkes Weber. An additional subgroup of unclassified associations is constituted by seven cases with bifocal intradural uni or multimetameric lesions. In our SCAVMs series, the incidence ofmultipie vascular lesions is high, in particular multifocal intradural malformations. Metameric distribution is the most frequent type of multiplicity. Identification of the myelomeric level involved in SCAVM allows segmental link between various lesions of mesodermal or neural crest origin to be discussed.

9 Rostrocaudal Differences within the Somites Confer Segmental Pattern to Trunk Neural Crest Migration

Neural crest cells are one of the most migratory and pleiotropic of embryonic cells types. These cells originate within the central nervous system, but emigrate from the neural tube shortly after its closure. They subsequently migrate along well characterized pathways to populate numerous and diverse derivatives ranging from sensory and autonomic ganglia of the peripheral nervous system to the cartilage and bone of the face. It is reported that neural crest migration is intimately linked to both the formation and segmentation of the somites. After differentiation of somites into dermomyotome and sclerotome, the sclerotomal compartment is subdivided into rostral and caudal halves. Although overtly similar, the caudal half sclerotome has a higher cell density and different molecular markers. Notably, ephrins (Eph) are selectively expressed in the caudal half sclerotome whereas their cognate Eph receptors are expressed on neural crest cells and rostral sclerotomal cells. Functional interactions between Eph receptors and ligands appear to restrict neural crest cells to the rostral half sclerotomal domain. This in turn leads to their segmental migration and the subsequent metameric distribution of neural crest-derived sensory and sympathetic ganglia.

The oldest arthropods of the East European Platform

Cassubia Lendzion 1977 and Liwia new name (for Livia Lendzion 1975 preoccupied) found in the subsurface Zawiszany formation of the basal Cambrian in NE Poland are identified as an anomalocarid and a ‘soft-bodied’ trilobite, respectively, each being the oldest representative of its group. The presence of four free thoracic tergites in Liwia, the relatively strong sclerotization, and the apparent segmentation of the pygidial shield (expressed in the metameric distribution of marginal spines) indicate that the ‘soft-bodied’ trilobites achieved their organization by secondary softening of the dorsal exoskeleton and suppression of the development of thoracic tergites in ontogeny.

Vertebral and epidural hemangioma with paraplegia in Klippel-Trenaunay-Weber syndrome

A patient with a rare case of Klippel-Trenaunay-Weber syndrome presented with paraplegia due to compression by a vertebral and epidural cavernous hemangioma. The metameric distribution of the large cutaneous vascular nevus provided the clinical clue to the nature of the spinal lesion. The association of the two lesions is explained in the basis of developmental anomaly.

Congenital Mixed Vascular Deformities of the Lower Limbs

Nineteen patients with vascular deformities of the lower extremity were studied and were classified into three groups based on their clinical and angiographic findings. 1) The Klippel or Venous Dysplasia Group (n = 8) demonstrated: cutaneous nevi of metameric distribution, congenital varices (many of which were anomalous by phlebography), hypertrophy of bone and other tissues, but no evidence of arterial involvement by arteriography. 2) Congenital Arterio-Venous Fistulae Group (n = 7) showed: cutaneous angiomata, hypertrophy of bone and soft tissues, but arteriographic findings of single or multiple arterio-venous fistulae. 3) Scattered Angiomata Group (n = 4) had single or multiple angiomata involving the lower limb in particular. Arteriography was normal, while phlbography revealed venous abnormalities. Lymphography was performed on all patients. Six of 8 of the Klippel group demonstrated aplastic or hypoplastic lymphatics by lymphography. By contrast, all 7 of the patients in the Congenital Arterio-Venous Fistulae Group showed in their lymphographic findings-three of 4 of these patients had aplasia or hypoplasia. Fourteen of the 19 patients complained of painful swelling as a major symptom. Lymphatic fistulae and lymphoceles developed in several patients.Four patients underwent reduction operations for lymphedema. Lymphatic abnormalities as demonstrated by lymphography play a significant role in mixed vascular deformities of the lower extremities.