Congenital neurological disorders

2018 ◽  
Author(s):  
Simon Rinaldi
Keyword(s):  
Cerebral Palsy ◽  
Spina Bifida ◽  
Neurological Disorders ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Foramen Magnum ◽  
Structural Relationships ◽  
Adult Medicine ◽  
Vertebral Arch ◽  
Chiari Malformations

This chapter covers four congenital neurological disorders which may be encountered in adult medicine: cerebral palsy, Chiari malformations, spina bifida, and tethered cord syndromes. Cerebral palsy is a disturbance of motor function arising from damage to the developing fetal or infant brain. It usually refers to a disorder resulting from a non-progressive insult which occurred at less than 3 years of age. Chiari malformations are congenital abnormalities of the anatomy and structural relationships of the cerebellum, the brainstem, and the foramen magnum. Dysraphism is a failure of opposition of anatomical structures which are normally fused. Spinal dysraphism is synonymous with spina bifida, a failure of embryological fusion of the neural tube. In all types, the vertebral arch fails to completely form. The tethered cord syndromes involve a restriction of the normal cephalad migration of the conus during life. This can occur both with and without spina bifida.

scholarly journals Spinal dysraphism with tripedus morphology: A case report

2021 ◽  
pp. 509-511
Author(s):  
Mohd Monis ◽  
Shagufta Wahab ◽  
Divyashree Koppal ◽  
Aiman Ibbrahim
Keyword(s):  
Case Report ◽  
Spina Bifida ◽  
Vertebral Column ◽  
Rare Case ◽  
Surgical Intervention ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Lower Back ◽  
Rare Case Report ◽  
Spinal Mri

This is a rare case report of a 5-month-old child with a complex spinal dysraphic state, and an accessory limb (tripedus morphology), accessory genitalia, and anal dimple. The child was brought to the hospital with an accessory limb arising from the back. On clinical examination, an accessory limb arising from the lower back with a partially developed foot with the presence of toes and nails was noted. Spinal MRI was advised which revealed dysraphic features including spina bifida with the low lying and posteriorly tethered cord with diastematomyelia along with a supernumerary appendage attached to the vertebral column having rudimentary bones resembling those of extremities. The presence of an accessory limb with spinal dysraphism is quite a rare anomaly. The condition can be treated by surgical intervention and involves excision of the accessory limb with adequate dural and paraspinal muscle cover.

Estimating the prevalence of neurosurgical interventions in adults with spina bifida using the Health Facts data set: implications for transition planning and the development of adult clinics

2021 ◽  
pp. 1-8
Keyword(s):  
Spina Bifida ◽  
Transition Planning ◽  
Care Delivery ◽  
Spinal Dysraphism ◽  
Temporal Trends ◽  
Tethered Cord ◽  
Neurosurgical Procedures ◽  
Coordinated Care ◽  
Data Set ◽  
Surgical Interventions

OBJECTIVE As the care of patients with spina bifida continues to evolve, life expectancy is increasing, leading to a critical need for transition planning from pediatric-based to adult-based care. The burden of neurosurgical care for adults with spina bifida remains unknown. In this study, the authors sought to use a large national data set to estimate the prevalence of neurosurgical interventions in adults with spina bifida. METHODS This study utilized Health Facts, which is a de-identified proprietary data set abstracted from all Cerner electronic health records. It includes 69 million unique patients with > 500 million encounters in 580 centers. Validation, technical exclusions, and data filters were applied to obtain an appropriate cohort of patients. The ICD-9 and ICD-10 codes for all types of spinal dysraphism, as well as the Current Procedural Terminology (CPT) codes for hydrocephalus procedures, spinal cord untethering, and Chiari decompression, were queried and records were retrieved. Demographic variables along with differences in age groups and temporal trends were analyzed. RESULTS Overall, 24,764 unique patients with ≥ 1 encounter with a spinal dysraphism diagnosis between 2000 and 2017 were identified. The pediatric cohort included 11,123 patients with 60,027 separate encounters, and the adult cohort included 13,641 patients with 41,618 separate encounters. The proportion of females was higher in the adult (62.9%) than in the pediatric (51.4%) cohort. Annual encounters were stable from 2 to 18 years of age, but then decreased by approximately half with a precipitous drop after age 21 years. The sex distribution of adults and children who underwent procedures was similar (54.6% female adults vs 52.4% female children). Surgical interventions in adults were common. Between 2013 and 2017, there were 4913 procedures for hydrocephalus, with 2435 (49.6%) adult patients. Similarly, 273 (33.3%) of the 819 tethered cord procedures were performed in adults, as were 307 (32.9%) of 933 Chiari decompressions. CONCLUSIONS The Health Facts database offered another option for studying care delivery and utilization in patients aging with spina bifida. The median age of this population has now reached early adulthood, and a significant number of neurosurgical procedures were performed in adults. An abrupt drop in the rate of encounters occurred at 21 years of age, possibly reflecting transition issues such as access-to-care problems and lack of coordinated care.

scholarly journals A rare case of thoracic myelocystocele associated with type 1 split cord malformation with low lying tethered cord, dorsal syrinx and sacral agenesis: Pentad finding

2015 ◽  
Vol 6 (01) ◽  
pp. 087-090 ◽  
Author(s):  
Dipanker Singh Mankotia ◽  
Guru Dutta Satyarthee ◽  
Bhawani Shankar Sharma
Keyword(s):  
Spina Bifida ◽  
Surgical Management ◽  
Rare Case ◽  
Clinical Presentation ◽  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Split Cord Malformation ◽  
Rare Form ◽  
Sacral Agenesis

ABSTRACTMyelocystocele is a rare form of spinal dysraphism. Thoracic myelocystocele is still rarer. The occurrence of thoracic myelocystocele associated with type-1 split cord malformation, low lying tethered cord, dorsal syrinx and spina bifida is extremely rare. Clinical presentation of such a rare case and an early surgical management is discussed briefly.

Chiari Malformations

2018 ◽  
Author(s):  
Marco Maurtua ◽  
Mathew Lyons ◽  
Nicholas DaPrano
Keyword(s):  
Spina Bifida ◽  
Autonomic Dysfunction ◽  
Spinal Canal ◽  
Foramen Magnum ◽  
Surgical Correction ◽  
Structural Defects ◽  
Neurologic Deficits ◽  
Base Of The Skull ◽  
The Brain ◽  
Chiari Malformations

Chiari malformations are structural defects in the base of the skull and cerebellum. These conditions are characterized by the abnormal displacement of part of the cerebellum and brainstem through the foramen magnum into the upper spinal canal causing autonomic dysfunction, neurologic deficits, and hydrocephalus. Chiari malformations are classified into several types based on their severity and the parts of the brain affected by the herniation. In neonates, Chiari malformations are commonly associated with spina bifida and myelomeningocele. Anesthesia for surgical correction of Chiari malformations presents a unique set of challenges and demands that requires knowledge of both the neurophysiology of the condition and the surgical process.

Data Mining in Analysis of Biomechanical Signals

2009 ◽  
Vol 147-149 ◽  
pp. 588-593 ◽  
Author(s):  
Marcin Derlatka ◽  
Jolanta Pauk
Keyword(s):  
Data Mining ◽  
Principal Component Analysis ◽  
Cerebral Palsy ◽  
Spina Bifida ◽  
Decision Tree ◽  
Principal Component ◽  
Data Preprocessing ◽  
Component Analysis ◽  
Kernel Principal Component Analysis

In the paper the procedure of processing biomechanical data has been proposed. It consists of selecting proper noiseless data, preprocessing data by means of model’s identification and Kernel Principal Component Analysis and next classification using decision tree. The obtained results of classification into groups (normal and two selected pathology of gait: Spina Bifida and Cerebral Palsy) were very good.

Lumbar spinal intradural extramedullary lipoma associated with spina bifida and tethered cord

2016 ◽  
Vol 16 (9) ◽  
pp. e611-e612 ◽  
Author(s):  
Zafer Orkun Toktaş ◽  
Baran Yılmaz ◽  
Murat Şakir Ekşi ◽  
Emin Değer ◽  
Deniz Konya ◽  
...  
Keyword(s):  
Spina Bifida ◽  
Tethered Cord ◽  
Intradural Extramedullary ◽  
Lumbar Spinal

scholarly journals The lumbosacral angle does not reflect progressive tethered cord syndrome in children with spinal dysraphism

2010 ◽  
Vol 26 (12) ◽  
pp. 1757-1764 ◽  
Author(s):  
Erwin M. J. Cornips ◽  
Femke G. E. M. Razenberg ◽  
Lodewijk W. van Rhijn ◽  
Dan L. H. M. Soudant ◽  
Elisabeth P. M. van Raak ◽  
...  
Keyword(s):  
Spinal Dysraphism ◽  
Tethered Cord ◽  
Tethered Cord Syndrome

What Do We Really Know About the Transition to Adult-Centered Health Care? A Focus on Cerebral Palsy and Spina Bifida

2007 ◽  
Vol 88 (8) ◽  
pp. 1064-1073 ◽  
Author(s):  
Jessie A. Binks ◽  
Wendy S. Barden ◽  
Tricia A. Burke ◽  
Nancy L. Young
Keyword(s):  
Health Care ◽  
Cerebral Palsy ◽  
Spina Bifida

EFFECTS OF ASPHYXIATION OF THE FETUS AND THE NEWBORN INFANT

PEDIATRICS ◽  
1960 ◽  
Vol 26 (4) ◽  
pp. 565-569
Author(s):  
William F. Windle
Keyword(s):  
Cerebral Palsy ◽  
Mental Retardation ◽  
Newborn Infant ◽  
Clinical Evaluation ◽  
Neurological Disorders ◽  
Basic Information ◽  
Clinical Experiences ◽  
Asphyxia Neonatorum

There is pressing need of well controlled clinical evaluation of the role of adverse factors in the prenatal, natal and early postnatal periods in the etiology of neurological disorders. This is particularly true of asphyxia neonatorum. Many articles have been written on relationships between apnea, anoxia or asphyxia and manifestations of mental retardation, cerebral palsy, epilepsy and othe neurological, psychological and behavioral defects. The majority of the writers express opinions, but give no evidence that data were collected. Controls are commonly lacking. Often no attempt to measure the variables appears to have been made. A review1 of more than 500 reports, commentaries and testimonials of clinical experiences reveals deficiency in basic information of physiology of the fetus and newborn that is nothing short of appalling.

scholarly journals Incidence of epilepsy among patients with cerebral palsy

2006 ◽  
Vol 41 (4) ◽  
pp. 202
Author(s):  
Pertin Sianturi ◽  
Amir Sarifuddin ◽  
Bistok Saing
Keyword(s):  
Cerebral Palsy ◽  
Neurological Disorders ◽  
Medical Records ◽  
Common Complication ◽  
Cerebral Function ◽  
Partial Seizures ◽  
Chi Square ◽  
Prolonged Seizure ◽  
High Incidence ◽  
Chi Square Test

Epilepsy is a chronic condition due to cerebral function disorders. Epilepsy occurs as a common complication of many neurological disorders such as cerebral palsy (CP) that can affect further brain damage especially if they are with prolonged seizure. The incidence of epilepsy among patients with CP varies between 25-35%. The high incidence of epilepsy among patients with CP suggests that this disorder has common or related origins. We carried out the retrospective study to determine incidence of epilepsy among patients with CP registered within July 1988 to June 1998 in YPAC Medan and to determine whether the incidence of epilepsy differed according to type of CP. Data were compiled from medical records, including name, sex, parity, mothers age, prenatal, perinatal, and postnatal history, and EEG results. Data were analyzed using statistical computer program and its significance was evaluated by chi square test at p < 0,05. There were 67 cases with CP, 53 cases spastic CP, 13 cases mixed CP and one case dyskinetic CP. Of the 67 cases CP, 47,8% male, 52,2% female and mean age 50,3 (SD 36,9) months. There were 25 (37,3%) patients CP associated with epilepsy, 72% general seizures, 20% partial seizures, and 8% infantile spasms. The incidence of epilepsy was significant different among patients with CP associated with type of CP and gestasional age, p < 0,05. We concluded that incidence of epilepsy among patients with CP in YPAC Medan was 37,3% and significantly different among patients with CP according to type CP and gestasional age.

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