Isolated sympathetic failure with autoimmune autonomic ganglionopathy.

Abstract:

:A 16-year-old boy had a gradual onset of post-exercise myalgia with progressive fatigue and dizziness. He had bradycardia (37 beats/minute) with low supine and normal standing norepinephrine levels (56 and 311 pg/mL, respectively). He had absent sympathetically mediated vasoconstrictor responses during Valsalva maneuver testing. Circulating ganglionic acetylcholine receptor antibodies were identified. Response was gradual to treatment with intravenous immunoglobulin combined with aggressive symptomatic interventions (permanent pacemaker implantation and treatment with pyridostigmine, midodrine, and modafinil). After the intravenous immunoglobulin treatment, his autoantibody levels decreased and the autonomic abnormalities resolved. After a reconditioning exercise program and eventually undetectable antibody titers, he achieved complete recovery. The patient continued to do well after his pacemaker was removed and his medications were discontinued. Thus, severe isolated sympathetic nervous system failure can occur in adolescents with autoimmune autonomic ganglionopathy, and multifaceted treatment can be effective.

journal_name

Pediatr Neurol

journal_title

Pediatric neurology

authors

Fischer PR,Sandroni P,Pittock SJ,Porter CB,Lehwald LM,Raj SR

doi

10.1016/j.pediatrneurol.2010.05.003

subject

Has Abstract

pub_date

2010-10-01 00:00:00

pages

287-90

issue

4

eissn

0887-8994

issn

1873-5150

pii

S0887-8994(10)00204-3

journal_volume

43

pub_type

杂志文章
  • Antiepileptic drug hypersensitivity syndrome: in vitro and clinical observations.

    abstract::Four patients who manifested symptoms of the antiepileptic drug (AED) hypersensitivity syndrome during therapy with carbamazepine are reported. In 3 patients, the syndrome was exacerbated after conversion of therapy to another antiepileptic drug with an aromatic ring chemical structure. In vitro lymphocyte transformat...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(94)90054-x

    authors: Alldredge BK,Knutsen AP,Ferriero D

    更新日期:1994-03-01 00:00:00

  • Transient Isolated Lower Bulbar Palsy With Elevated Serum Anti-GM1 and Anti-GD1b Antibodies During Aripiprazole Treatment.

    abstract:BACKGROUND:Transient bulbar palsy without involvement of the facial or extraocular muscles is a rare presentation. It is considered a form of cranial polyneuropathy, a variant of Guillain-Barré syndrome that is related to the autoimmune mechanisms induced by preceding infections or vaccinations. However, drug-induced c...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2016.07.011

    authors: Han TH,Kim DY,Park DW,Moon JH

    更新日期:2017-01-01 00:00:00

  • Clinical features of developmental disability associated with cerebellar hypoplasia.

    abstract::Sporadic nonsyndromic cerebellar hypoplasia is a radiological diagnosis with clinical features and a relation with developmental disability that are presently not known. Through a retrospective review of a comprehensive standardized computerized database containing more than 2,500 patients examined consecutively by a ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(96)00220-2

    authors: Shevell MI,Majnemer A

    更新日期:1996-10-01 00:00:00

  • The Unique Coexistence of Anti-SS-A/Ro Antibodies in a Neonate with Symptomatic Ischemic Stroke.

    abstract:BACKGROUND:Neonatal cerebral infarction is a relatively common cause of neonatal seizures, with an incidence of at least 1:4000 live births and is associated with a high incidence of neurological sequelae. However, the pathophysiological mechanisms and predisposing factors responsible for neonatal infarction are not fu...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2016.06.005

    authors: Kanda K,Sato A,Abe D,Nishijima S,Ishigami T

    更新日期:2016-09-01 00:00:00

  • Developing the Brain-Early Illustrations of Cerebral Cortex and Its Gyri.

    abstract:BACKGROUND:Throughout the Middle Ages, most representations of the brain amounted to highly schematized ventricles housed within abstract squiggles of neural tissue. The works by the pre-eminent Flemish anatomist Andreas Vesalius in his De Humani Corporis Fabrica (1543) added considerably more accuracy and detail; stil...

    journal_title:Pediatric neurology

    pub_type: 历史文章,杂志文章,评审

    doi:10.1016/j.pediatrneurol.2017.07.004

    authors: Sutherland-Foggio H

    更新日期:2017-10-01 00:00:00

  • Postneonatal epilepsy following amplitude-integrated EEG-detected neonatal seizures.

    abstract::To assess the incidence of postneonatal epilepsy in term infants treated with antiepileptic drugs for neonatal seizure discharges that were detected with amplitude-integrated electroencephalography (aEEG), 206 term infants were monitored using this modality. They received antiepileptic drugs for clinical as well as su...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2004.11.005

    authors: Toet MC,Groenendaal F,Osredkar D,van Huffelen AC,de Vries LS

    更新日期:2005-04-01 00:00:00

  • SPECT in focal enterovirus encephalitis: evidence for local cerebral vasculitis.

    abstract::We report a 4-year-old, left-handed male with focal coxsackievirus A3 encephalitis who presented with seizures and acquired aphasia. Electroencephalography exhibited focal spike discharges over the right frontal regions, but cranial magnetic resonance imaging did not reveal any structural abnormalities. However, brain...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(00)00206-x

    authors: Wakamoto H,Ohta M,Nakano N,Kunisue K

    更新日期:2000-11-01 00:00:00

  • Efficacy of lamotrigine in refractory neonatal seizures.

    abstract::A newborn infant with seizures of unknown etiology that were refractory to treatment with phenobarbitone, phenytoin, midazolam, clonazepam, and vigabatrin is reported. The introduction of the new antiepileptic drug lamotrigine was followed by rapid and sustained control of the seizures. ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(98)00125-8

    authors: Barr PA,Buettiker VE,Antony JH

    更新日期:1999-02-01 00:00:00

  • Neonatal Brain Microstructure and Machine-Learning-Based Prediction of Early Language Development in Children Born Very Preterm.

    abstract:BACKGROUND:Very-low-birth-weight preterm infants have a higher rate of language impairments compared with children born full term. Early identification of preterm infants at risk for language delay is essential to guide early intervention at the time of optimal neuroplasticity. This study examined near-term structural ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2020.02.007

    authors: Vassar R,Schadl K,Cahill-Rowley K,Yeom K,Stevenson D,Rose J

    更新日期:2020-07-01 00:00:00

  • Acute necrotizing encephalopathy associated with hemophagocytic syndrome.

    abstract::A 7-year-old female suddenly exhibited high fever and convulsions, and entered a semi-coma. She also had thrombocytopenia, elevated aminotransferase, prolonged prothrombin time and activated partial thromboplastin time, and hemophagocytes in the bone marrow. The brain magnetic resonance imaging revealed multiple low-i...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2005.08.030

    authors: Akiyoshi K,Hamada Y,Yamada H,Kojo M,Izumi T

    更新日期:2006-04-01 00:00:00

  • Choreoathetosis after cardiac surgery with hypothermia and extracorporeal circulation.

    abstract::Eleven children, 4-48 months old, with congenital cyanotic heart defects developed choreoathetoid movements 2-12 days after cardiac surgery with hypothermia and extracorporeal circulation (ECC). The abnormal movements mainly involved the limbs, facial musculature, and tongue, leading to a severe dysphagia. The symptom...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(98)00036-8

    authors: Gherpelli JL,Azeka E,Riso A,Atik E,Ebaid M,Barbero-Marcial M

    更新日期:1998-08-01 00:00:00

  • Frontal white matter reductions in healthy males with complex stereotypies.

    abstract::The pathophysiologic mechanism for stereotypic, bilateral repetitive movements involving the arms and hands (complex motor stereotypies) is unknown. This study used volumetric magnetic resonance imaging to compare cerebral lobes and caudate nucleus in six males with complex stereotypies and average intelligence to age...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2004.09.005

    authors: Kates WR,Lanham DC,Singer HS

    更新日期:2005-02-01 00:00:00

  • Sixth nerve palsies in children.

    abstract::The causes of sixth nerve palsies in 75 children, all of whom had undergone modern neuroimaging, were reviewed. Neoplasms or their neurosurgical removal was the most common cause (n = 34 [45%]); elevated intracranial pressure (nontumor) (15%), traumatic (12%), congenital (11%), inflammatory (7%), miscellaneous (5%), a...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/s0887-8994(98)00090-3

    authors: Lee MS,Galetta SL,Volpe NJ,Liu GT

    更新日期:1999-01-01 00:00:00

  • Acute disseminated encephalomyelitis with probable measles vaccine failure.

    abstract::The patient is a 10-year-old male who experienced somnolence and incomplete quadriplegia after headache and vomiting, without exanthema, for 3 days. The clinical course and magnetic resonance imaging findings of the brain and spinal cord were compatible with acute disseminated encephalomyelitis. The serologic examinat...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(99)00005-3

    authors: Nagai K,Mori T

    更新日期:1999-05-01 00:00:00

  • Neuromyelitis optica in an adolescent after bone marrow transplantation.

    abstract:BACKGROUND:Central nervous system complications of bone marrow transplant are a common occurrence and the differential diagnosis is quite broad, including opportunistic infections, medications toxicities, graft versus host disease, and other autoimmune processes. PATIENT DESCRIPTION:We summarize previously reported ca...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pediatrneurol.2014.07.007

    authors: Baumer FM,Kamihara J,Gorman MP

    更新日期:2015-01-01 00:00:00

  • Rapid eye movement latency in children and adolescents.

    abstract::Rapid eye movement sleep distribution changes during development, but little is known about rapid eye movement latency variation in childhood by age, sex, or pathologic sleep states. We hypothesized that: (1) rapid eye movement latency would differ in normal children by age, with a younger cohort (1-10 years) demonstr...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2008.06.011

    authors: Mason TB 2nd,Teoh L,Calabro K,Traylor J,Karamessinis L,Schultz B,Samuel J,Gallagher PR,Marcus CL

    更新日期:2008-09-01 00:00:00

  • Rolandic mitochondrial encephalomyelopathy and MT-ND3 mutations.

    abstract::Mitochondrial encephalopathies may be caused by mutations in the respiratory chain complex I subunit genes. Described here are the cases of two pediatric patients who presented with MELAS-like calcarine lesions in addition to novel, bilateral rolandic lesions and epilepsia partialis continua, secondary to MT-ND3 mutat...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2009.02.010

    authors: Werner KG,Morel CF,Kirton A,Benseler SM,Shoffner JM,Addis JB,Robinson BH,Burrowes DM,Blaser SI,Epstein LG,Feigenbaum AS

    更新日期:2009-07-01 00:00:00

  • Tyrosine hydroxylase deficiency in Taiwanese infants.

    abstract::We analyzed the clinical manifestations, genetic mutations, treatment responses to L-dopa, and long-term neurologic outcomes in Taiwanese infants with tyrosine hydroxylase deficiency. From 1999 to May 2011, we enrolled six infants who had been diagnosed with tyrosine hydroxylase deficiency by identifying point mutatio...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2011.11.012

    authors: Chi CS,Lee HF,Tsai CR

    更新日期:2012-02-01 00:00:00

  • Seizures in a 7-month-old child after exposure to the essential plant oil thuja.

    abstract::A previously healthy 7-month-old child was treated with homeopathic preparations of thuja, a potentially convulsant compound, for the purpose of providing a calming effect around times of immunizations. The child developed eight generalized tonic-clonic seizures with no other obvious cause, in the context of normal el...

    journal_title:Pediatric neurology

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.pediatrneurol.2007.07.008

    authors: Stafstrom CE

    更新日期:2007-12-01 00:00:00

  • Wide cavum septum pellucidum: a marker of disturbed brain development.

    abstract::A wide cavum septum pellucidum defined as a separation of greater than 1 cm of the leaves occurs uncommonly. Nine children with wide cavum septum pellucidum were studied; 8 were abnormal. Observed abnormalities included cognitive impairment (8), seizures (4), hypoplasia of the corpus callosum (4), optic nerve hypoplas...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(90)90007-n

    authors: Bodensteiner JB,Schaefer GB

    更新日期:1990-11-01 00:00:00

  • West syndrome following deep hypothermic infant cardiac surgery.

    abstract::Postoperative seizures are among the more common complications of cardiac surgery in children. These seizures have traditionally been considered benign, transient phenomena with little, if any, prognostic significance. We report 4 infants with early postoperative seizures following cardiac surgery who later developed ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(94)90111-2

    authors: du Plessis AJ,Kramer U,Jonas RA,Wessel DL,Riviello JJ

    更新日期:1994-10-01 00:00:00

  • Correlation Among Genotype, Phenotype, and Histology in Neuronal Ceroid Lipofuscinoses: An Individual Patient Data Meta-Analysis.

    abstract:BACKGROUND:Neuronal ceroid lipofuscinoses (NCL) are heterogeneous neurodegenerative disorders. A better understanding of genotype-phenotype-histology correlation is expected to improve patient care and enhance understanding for phenotypic variability. This meta-analysis studies the correlation of NCL genotypes with cli...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,meta分析,评审

    doi:10.1016/j.pediatrneurol.2016.03.018

    authors: Aungaroon G,Hallinan B,Jain P,Horn PS,Spaeth C,Arya R

    更新日期:2016-07-01 00:00:00

  • Parental attitude of mothers, iron deficiency anemia, and breath-holding spells.

    abstract::The aim of this study was to investigate the effect of family structure and the attitude of mothers on the development of breath-holding spells. The data of the Sociodemographic and Parental Attitude Research Instruments of 30 mothers of children with breath-holding spells and of 30 mothers of healthy children were co...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2005.12.006

    authors: Hüdaoglu O,Dirik E,Yiş U,Kurul S

    更新日期:2006-07-01 00:00:00

  • Methotrexate leukoencephalopathy presenting as Klüver-Bucy syndrome and uncinate seizures.

    abstract::Methotrexate causes several biochemical changes that impact the nervous system. The neurotoxicity usually affects the cerebral white matter, causing a leukoencephalopathy that can be chronic and progressive with cognitive decline. A 15-year-old male developed olfactory seizures and behavioral abnormalities (hypersexua...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/s0887-8994(01)00376-9

    authors: Antunes NL,Souweidane MM,Lis E,Rosenblum MK,Steinherz PG

    更新日期:2002-04-01 00:00:00

  • Phasic sleep components in infants with cyanosis during feeding.

    abstract::Although brainstem immaturity has been postulated as one of the pathogenesis underlying cyanosis during feeding (CDF), there has been no widely accepted physiologic parameter that reflects brainstem function. We recently proposed that the dissociation index (DI), one of the phasic sleep parameters, is a reliable and q...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(91)90085-y

    authors: Kohyama J,Watanabe S,Iwakawa Y

    更新日期:1991-05-01 00:00:00

  • Cerebrospinal fluid TNF-α, IL-6, and IL-8 in children with bacterial meningitis.

    abstract:OBJECTIVE:We evaluated the levels of cerebrospinal fluid concentrations of tumor necrosis factor-α, interleukin-6, and interleukin-8 in bacterial meningitis in children. METHODS:The study included children up to 14 years of age admitted to a pediatric ward with fever, headache, vomiting, and seizures. The diagnosis of...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/j.pediatrneurol.2013.08.016

    authors: Prasad R,Kapoor R,Srivastava R,Mishra OP,Singh TB

    更新日期:2014-01-01 00:00:00

  • Acute Disseminated Encephalomyelitis in Children: An Updated Review Based on Current Diagnostic Criteria.

    abstract::Acute disseminated encephalomyelitis is an inflammatory disorder of the central nervous system. Uniform diagnostic criteria for acute disseminated encephalomyelitis did not exist until publication of expert-defined consensus definitions by the International Pediatric Multiple Sclerosis Society Group in 2007, with upda...

    journal_title:Pediatric neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.pediatrneurol.2019.06.017

    authors: Cole J,Evans E,Mwangi M,Mar S

    更新日期:2019-11-01 00:00:00

  • Pregabalin: preliminary experience in intractable childhood epilepsy.

    abstract::Pregabalin is a new antiepileptic drug that acts at presynaptic calcium channels, modulating neurotransmitter release. We report on treating consecutive children with severe drug-resistant epilepsy in a prospective, open-label, add-on trial. Nineteen children (63% male) aged 4-15 years (mean, 9.7; S.D., 2.9) were incl...

    journal_title:Pediatric neurology

    pub_type: 临床试验,杂志文章

    doi:10.1016/j.pediatrneurol.2008.12.016

    authors: Jan MM,Zuberi SA,Alsaihati BA

    更新日期:2009-05-01 00:00:00

  • Benign hereditary chorea improved on stimulant therapy.

    abstract::Stimulant therapy is usually contraindicated in patients with movement disorders such as tics or chorea. A young boy is reported who had benign hereditary chorea and attention deficit disorder, whose chorea, handwriting, and independent ambulation paradoxically improved with methylphenidate treatment. ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(96)00056-2

    authors: Friederich RL

    更新日期:1996-05-01 00:00:00

  • Epidemiology of bacterial meningitis in children: Aichi Prefecture, Japan, 1984-1993.

    abstract::The details of 328 patients with bacterial meningitis, admitted from 1984 through 1993, were obtained from 46 departments of pediatrics of large hospitals through questionnaires. The incidence rate per 100,000 child-years was 2.32, being higher in children aged 0-4 years (rate, 7.22) than 5-15 years (rate, 0.49). The ...

    journal_title:Pediatric neurology

    pub_type: 杂志文章

    doi:10.1016/0887-8994(96)00024-0

    authors: Ishikawa T,Asano Y,Morishima T,Nagashima M,Sobue G,Watanabe K,Yamaguchi H

    更新日期:1996-04-01 00:00:00