ABSTRACT
Neuromuscular blocking agents are routinely used as an adjunct therapy for critically ill patients. Unlike many neuromuscular blocking agents, vecuronium does not cause significant histamine release, which may lead to bronchoconstriction. Due to a short duration of action and limited accumulation, vecuronium has been widely used. Prolonged ventilatory dependence due to persistent neuromuscular blockade has been reported in patients treated with vecuronium. We report a case of an 8-year-old girl who had a primarily motor axonopathy presenting with weakness after extended vecuronium administration with prolonged course of recovery. This primarily motor neuropathy with axonal features may be a variant of critical illness polyneuropathy, a rarely reported condition in pediatric patients.
Subject(s)
Motor Neuron Disease/chemically induced , Motor Neuron Disease/diagnosis , Neuromuscular Nondepolarizing Agents/adverse effects , Polyneuropathies/chemically induced , Polyneuropathies/diagnosis , Vecuronium Bromide/adverse effects , Child , Electromyography , Female , Humans , Motor Neuron Disease/classification , Neural Conduction/drug effects , Polyneuropathies/classification , Recovery of FunctionABSTRACT
Polyneuropathies are relatively uncommon in early infancy and the majority of affected children are found to have hypomyelinating neuropathies. Axonal sensorimotor neuropathies have been described in childhood but the majority of affected children present at or after 6 months of age, have nonprogressive courses, and achieve the ability to walk, albeit late. Here we present three infants with infantile progressive axonal polyneuropathy from two families with nonconsanguineous parents. Each child presented shortly after the neonatal period and with rapid progression to quadriplegia. Involvement of the lower cranial nerves, phrenic nerves, or both was present in each child. Electrophysiology was diagnostic in each child. While the diagnosis of spinal muscular atrophy was considered in each case, clinical presentation, biopsies, and genetic testing were inconsistent with this diagnosis. Recognition of this early form of progressive axonal neuropathy is important as respiratory compromise occurred early and the condition showed familial inheritance in two of our patients.
Subject(s)
Hereditary Sensory and Motor Neuropathy/complications , Muscle, Skeletal/pathology , Spinal Cord/pathology , Sural Nerve/pathology , Age of Onset , Axons/pathology , Biopsy , Electrophysiology , Fatal Outcome , Hereditary Sensory and Motor Neuropathy/diagnosis , Hereditary Sensory and Motor Neuropathy/physiopathology , Humans , Infant , Male , Muscle, Skeletal/innervation , Quadriplegia/etiology , Respiration, Artificial , Respiratory Insufficiency/etiologyABSTRACT
In children, posterior fossa juvenile pilocytic astrocytomas are common tumors. Radiologically, primarily extra-axial brainstem pilocytic astrocytomas are uncommon and extra-axial cerebellar pilocytic tumors are exceptional. We report two cases of such exophytic tumors, contrasting their presentation, imaging appearance, and prognosis. We also report the radiographic features of posterior fossa juvenile pilocytic astrocytomas from a 5-year review of our institutional experience.
Subject(s)
Astrocytoma/diagnosis , Infratentorial Neoplasms/diagnosis , Astrocytoma/pathology , Child , Child, Preschool , Diagnosis, Differential , Female , Humans , Infratentorial Neoplasms/pathology , Magnetic Resonance Imaging , Male , Prognosis , Tomography, X-Ray ComputedSubject(s)
Charcot-Marie-Tooth Disease/complications , Charcot-Marie-Tooth Disease/genetics , Chromosomes, Human, Pair 5 , Glycogen Storage Disease Type V/complications , Glycogen Storage Disease Type V/genetics , Membrane Proteins , Nerve Tissue Proteins , Trisomy , Adult , Charcot-Marie-Tooth Disease/diagnosis , Child , Female , Glycogen Storage Disease Type V/diagnosis , Humans , Male , Myelin and Lymphocyte-Associated Proteolipid Proteins , Proteolipids/geneticsABSTRACT
The clinical course of a 6-year-old boy with adrenoleukodystrophy (ALD) who underwent allogeneic stem-cell transplantation during an early clinical stage is described. Twenty-three months after transplant, he remains neurologically stable, but with moderate neurological sequelae; the serum very long chain fatty acid profile has improved, but not normalized. The indications, mechanism of action, and complications of bone marrow transplantation in ALD are discussed briefly, along with other potential therapies.
Subject(s)
Adrenoleukodystrophy/complications , Adrenoleukodystrophy/genetics , Learning Disabilities/diagnosis , Reading , Acyl Coenzyme A/blood , Adrenoleukodystrophy/therapy , Brain/pathology , Brain/surgery , Child , Combined Modality Therapy , Humans , Intellectual Disability/diagnosis , Learning Disabilities/etiology , Magnetic Resonance Imaging , Male , Stem Cell Transplantation , Wechsler Scales , X Chromosome/geneticsABSTRACT
Carbon monoxide (CO) is one of the common inhaled intoxicants used in attempting suicide. This case report describes the multidisciplinary rehabilitation management of suicidal CO poisoning in a 13-year-old initially comatose male patient. Despite the prevalence of self-inflicted CO poisoning, this is the first detailed case report emphasizing the acute and delayed neuropsychiatric sequelae of CO poisoning in the premorbidly depressed patient. In addition, the difficulty of assessing psychiatric illness in an acutely neurologically impaired patient is discussed.
Subject(s)
Brain Injuries/pathology , Carbon Monoxide Poisoning , Depression/rehabilitation , Patient Care Team , Adolescent , Brain Injuries/chemically induced , Depression/etiology , Humans , Male , Suicide, AttemptedABSTRACT
Neurofibromatosis 1 is a common, genetically transmitted neurodevelopmental disorder with a high potential to cause subcortical focal brain lesions. Although seizures occasionally complicate neurofibromatosis 1, they have not been characterized adequately in the disease. Other phacomatoses with attendant subcortical focal brain lesions have been associated with an evolution from generalized to focal-onset epilepsy. This evolution may be related to the cortical dysplasias that also appear in such disorders. Cortical dysplasias, although occasionally found in neurofibromatosis 1 epileptic patients, are not as frequent as in other phacomatoses. The authors retrospectively screened 499 neurofibromatosis 1 patients for the prevalence and etiology of seizures, their response to therapy, and the evolution of seizure type over time. The prevalence of epileptic seizures (4.2%) was about twice the prevalence in the general population. There were no significant cortical dysplasias identified radiographically nor was there a relationship among the presence of subcortical focal brain lesions and seizure type, response to treatment, or evolution of epilepsy in the neurofibromatosis 1 population. The authors' data do not suggest an ontogeny of epilepsy in neurofibromatosis 1 that is different from the general epileptic population.
Subject(s)
Neurofibromatosis 1/complications , Seizures/etiology , Adolescent , Adult , Child , Child, Preschool , Cross-Sectional Studies , Electroencephalography , Epilepsy/complications , Epilepsy/diagnosis , Epilepsy/epidemiology , Epilepsy/etiology , Female , Humans , Infant , Infant, Newborn , Magnetic Resonance Imaging , Male , Neurofibromatosis 1/diagnosis , Prevalence , Retrospective Studies , Seizures/diagnosis , Seizures/epidemiology , Tomography, X-Ray ComputedABSTRACT
We report the case of an infant with Menkes' disease who presented with moderately abnormal neurologic findings at birth and with extraaxial bleeding in the posterior fossa. Early cerebellar atrophy and hypomyelination were evident on magnetic resonance imaging at 5 weeks of age. We suggest that neurodegeneration occurs earlier than has been previously reported and may be identifiable even in neonates.
Subject(s)
Cerebral Hemorrhage/diagnosis , Magnetic Resonance Imaging , Menkes Kinky Hair Syndrome/diagnosis , Nerve Degeneration/diagnosis , Tomography, X-Ray Computed , Humans , Infant , MaleABSTRACT
Sarcomas (chondrosarcoma, Ewing sarcoma, rhabdomyosarcoma, and undifferentiated sarcoma) generally present with nonneurologic symptoms at the onset. Five children with sarcomas who presented with spinal cord compression or radiculopathy as their initial problems are described. These patients appear to be older than those with the more commonly encountered neuroblastoma-related spinal cord compression. Our patients had a favorable neurologic recovery initially, but ultimately succumbed to systemic complications from spread of the sarcoma.
Subject(s)
Sarcoma/diagnosis , Spinal Cord Compression/etiology , Spinal Nerve Roots/pathology , Adolescent , Child , Female , Humans , Male , Retrospective Studies , Sarcoma/complicationsABSTRACT
A child with acute lymphocytic leukemia who was in clinical remission and developed coxsackie B4 meningoencephalitis is presented. Despite minimal evidence of immunodeficiency, the patient had progression of neurologic disease clinically, electroencephalographically and on laboratory evaluation of spinal fluid. A dramatic response to the use of intravenous immunoglobulins is reported and its rationale explained.
Subject(s)
Coxsackievirus Infections/therapy , Immunocompromised Host , Immunoglobulins, Intravenous/therapeutic use , Meningoencephalitis/therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/immunology , Child, Preschool , Coxsackievirus Infections/immunology , Humans , Male , Meningoencephalitis/immunology , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapyABSTRACT
Many investigators have identified localized cortical involvement in subacute sclerosing panencephalitis (SSPE) by clinical and electrophysiologic criteria. Some investigators have reported such abnormalities in the posterior cerebrum early in the course of the disease, but without radiologic correlation. Recently, magnetic resonance imaging has been used to follow the progression of SSPE. The largest reported study of SSPE utilizing magnetic resonance imaging indicated that focal abnormalities were distinctly rare and actually mitigated against the diagnosis of SSPE. We report an SSPE patient with focal cerebral dysfunction and magnetic resonance imaging evidence of distinctly focal inflammatory disease early in the course of the illness.