Authors

Michael V Johnston MD

Profile

Professor Emeritus of Neurology and Pediatrics
Johns Hopkins University School of Medicine
Former Chief Medical Officer & Blum Moser Endowed Chair for Pediatric Neurology
Kennedy Krieger Institute/Hugo Moser Research Institute

Founding Editorial Board Member since 1993: Pediatric Neurology

Clinical Interests: Child neurology

Research Interests: Therapies to reduce brain injury in infants and children as well as to promote recovery by enhancing brain plasticity

Contributor Disclosures

Dr. Johnston of Johns Hopkins University School of Medicine has no relevant financial relationships to disclose.

Developmental Malformations

Vein of Galen malformations

Vein of Galen malformations are congenital arteriovenous malformations involving the deep venous system of the brain that were first described in 1895

Sep. 24, 2021
General Child Neurology

Neonatal white matter injury

Neonatal white matter injury is the leading cause of cerebral palsy and other neurocognitive deficits in preterm-born children. White matter injury is the result of the selective vulnerability of pre-oligodendrocytes in the preterm brain to hypoxia-ischemia. This vulnerability may be potentiated by inflammation. White matter injury severity and location are predictors of adverse neurodevelopmental outcomes.

Jul. 12, 2021
General Child Neurology

Infant botulism

Infant botulism is a serious bacterial infection that results from ingestion of Clostridium botulinum spores that colonize the intestinal tract, releasing botulinum toxin and resulting in progressive neuromuscular weakness. It presents with a progression of symptoms: constipation, cranial nerve palsies, flaccid descending paralysis, and possible respiratory failure. Treatment includes urgent administration of botulism immune globulin (BabyBIG®) as well as supportive care.

May. 31, 2021
General Child Neurology

Nondominant hereditary ataxias

Hereditary ataxias are characterized by degeneration of the cerebellum and spinal cord. Friedreich ataxia (FRDA), caused by a GAA trinucleotide (triplet) repeat expansion mutation, is the most common nondominant form. A childhood onset spinocerebellar ataxia (SCA), FRDA is associated with scoliosis, cardiomyopathy, and absent knee and ankle jerks.

Apr. 16, 2021
Behavioral & Cognitive Disorders

Developmental language disorder

A developmental language disorder is a primary disability, with no comorbidities such as sensory impairment, intellectual disability, global developmental delay, motor dysfunction, autism spectrum disorder, or attention deficit hyperactivity disorder. It is a relatively common developmental finding in children and has some long-term impacts. No clear etiology has been determined, but there is much interest in finding a gene or genes that affect language development.

Apr. 16, 2021
General Child Neurology

Acute cerebellar ataxia in children

Acute cerebellar ataxia in children typically presents with sudden onset of unsteadiness, gait difficulties, and nystagmus, usually following an acute viral illness or vaccination. Headache, photophobia, and lightheadedness may also occur. Varicella, Epstein-Barr virus, influenza, and mycoplasma can be causes.

Apr. 05, 2021
General Child Neurology

Cerebral palsy

Cerebral palsy is characterized by abnormalities in tone, posture, and movement, and associated impairments including motor dysfunction, language delay, seizures, strabismus, dysphagia, orthopedic deformities, and cognitive problems. It represents a group of nonprogressive genetic and acquired childhood neurologic disorders that occur as a result of early injury to the developing brain.

Apr. 05, 2021
Behavioral & Cognitive Disorders

Angelman syndrome

Angelman syndrome is a neurodevelopmental disorder characterized by intellectual disability, epilepsy, ataxia, and a unique behavioral phenotype. It has generally been felt to result from impaired expression of the maternal allele for the UBE3A gene, which encodes ubiquitin protein ligase E3A. Primary areas of clinical management include the following: seizures, sleep, aspiration risk, GERD, constipation, dental care, vision, obesity, scoliosis, bone density, mobility, communication, behavior, and anxiety.

Apr. 05, 2021

Michael V Johnston MD

Contributor Disclosures

Related Articles

Vein of Galen malformations

Neonatal white matter injury

Infant botulism

Nondominant hereditary ataxias

Developmental language disorder

Acute cerebellar ataxia in children

Cerebral palsy

Angelman syndrome