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Guillain-Barré syndrome is characterized by acute progressive weakness, areflexia, and maximal motor disability that occur within 4 weeks of onset. Various clinical subtypes have been described since the original description of the syndrome. This study aimed to identify characteristics of clinical variants of Guillain-Barré syndrome through retrospective review of cases in Chang Gung Children's Hospital from 2000-2010. Forty-three Guillain-Barré syndrome patients were evaluated based on clinical presentations and an electrodiagnostic study. The most frequent variant of Guillain-Barré syndrome was demyelinating polyneuropathy (67.4%), followed by acute axonal neuropathy (7.0%), Miller Fisher syndrome (7.0%), Bickerstaff brainstem encephalitis (7.0%), pharyngo-cervical-brachial variant (4.7%), and polyneuritis cranialis (4.7%). Follow-up revealed that 35 recovered satisfactorily, eight were persistently disabled, and none died during hospitalization. At the earliest stage, differentiating clinical variants from typical Guillain-Barré syndrome was difficult. Children with clinical variants of Guillain-Barré syndrome are more likely to manifest rapid onset from disease onset to nadir, increasing the severity of disability, cranial nerve involvement, urine incontinence, respiratory impairment, and need for ventilator support than in typical Guillain-Barré syndrome.

Guillain-Barré syndrome and acute myelitis represent common and very rare etiologies of acute limb weakness in children, respectively. Typically, demyelination is most common, limited to either the central or peripheral nervous system. Concurrent acute myelitis and Guillain-Barré syndrome are seldom reported. We retrospectively reviewed cases of Guillain-Barré syndrome in Chang Gung Children's Hospital from 2000-2008. The risk factors of Guillain-Barré syndrome with or without acute myelitis were analyzed. Thirty-six children with Guillain-Barré syndrome were enrolled. Five children manifested coexisting acute myelitis. In the disease's earliest stage, differentiating concurrent acute myelitis and Guillain-Barré syndrome from Guillain-Barré syndrome only was difficult. Four of 5 children were aged more than 8 years. All patients developed a sharply defined sensory level, and manifested severe bladder dysfunction. Autonomic dysfunction was evident in three patients. All children with sufficient follow-up continued to demonstrate residual paresis, and two demonstrated significant long-term motor deficits. Detection of a sharply defined sensory level, severe bladder dysfunction, and autonomic dysfunction can be helpful in recognizing concurrent acute myelitis. Therefore, recognition of this combination of peripheral and central nervous inflammation early is important for determining prognoses and perhaps initial treatment.

Encephalitis is characterized clinically by fever, seizure, and an altered level of consciousness. Mycoplasma pneumoniae, a common respiratory pathogen, has been implicated as an etiology of encephalitis. The present study was designed to analyze status epilepticus associated with M. pneumoniae encephalitis in a series of children through retrospective review of cases between January 2002 and January 2008. Systematic clinical data were evaluated. Nine patients were identified: five girls and four boys, aged 4 years to 10 years. All were positive for M. pneumoniae by serology. Six of the nine children (67%) developed refractory status epilepticus. The major clinical symptoms included fever (100%) and upper respiratory symptoms (78%). The most common seizure type was primary focal with secondary generalized seizure (44%). The time of follow-up for this study ranged from 18 months to 86 months. At the end of the study period, two patients had died, seven had developed epilepsy or neurologic deficits, and none had returned to baseline. These data indicate that children with status epilepticus associated with M. pneumoniae encephalitis have high mortality and morbidity. Clinicians should be aware of the potential role of M. pneumoniae in status epilepticus.

Despite advances in critical care, febrile infection-related epilepsy syndrome remains the most important cause of mortality and neurologic deficits during childhood. Only a few therapeutic agents were reported to shorten the acute phase and improve outcomes. Therapeutic hypothermia was reported effective in stabilizing immune activation, brain edema, and seizure activity, to protect the brain from ongoing functional, apoptotic neural, and glial damage and the systemic expansion of the cytokine storm. We present two pediatric cases of febrile infection-related epilepsy syndrome, refractory to conventional medical therapy. Moderate therapeutic hypothermia at 33°C resulted in fast, sustained control of refractory status epilepticus. After 3 months, both patients recovered with a Glasgow Outcome Scale score of 4. Therapeutic hypothermia may play an important role in children with febrile infection-related epilepsy syndrome.

Background A ketogenic diet has been used successfully to treat intractable epilepsy. However, the role of early intravenous initiation of ketogenic diet in the acute phase of super-refractory status epilepticus is not well-described. Methods An intravenous ketogenic diet was administered to a boy with super-refractory status epilepticus. At 24 hours after intravenous ketogenic diet, moderate ketosis appeared, and thiamylal was successfully weaned at 70 hours after admission. Results An intravenous ketogenic regimen led to subsequent ketosis and seizure control in a child with super-refractory status epilepticus. Conclusion Early induction of ketosis may be a novel strategy to effectively treat super-refractory status epilepticus. Although there are few data regarding the early use of intravenous ketogenic diet in the treatment of super-refractory status epilepticus, it may be considered an alternative option.

Pneumocephalus is uncommon and usually evident after head trauma or surgery. It is a rare complication of acute, isolated sphenoid sinusitis caused by fungus pathogens in an immunocompetent patient. We report on a 14-year-old healthy boy with acute, isolated sphenoid sinusitis caused by Candida albicans. Pneumocephalus was the initial overlooked presentation. Although uncommon, fungal disease should be considered in acute, isolated sphenoid sinusitis when pneumocephalus is discovered, even in immunocompetent patients. Failure to diagnose and treat this entity promptly usually results in rapid progression and death.

Wilson disease is an autosomal recessive inborn error of copper metabolism that leads to neurologic symptoms and variable degrees of hepatic damage. The most common characteristic signs clinically are liver disease, psychiatric disease, neurologic disease, or a combination of these. Early recognition by means of clinical signs and an early initiation of therapy using chelators or zinc-salts are essential for a good outcome and prognosis. This report describes a male suffering from Wilson disease who exhibited an unusual presentation that included psychological manifestations without hepatic involvement. He was initially treated for attention-deficit hyperactivity disorder and a seizure disorder until brain imaging established the diagnosis of Wilson disease.

Since the discovery of antibodies against the N-methyl-D-aspartate receptor in 2007, anti-N-methyl-D-aspartate receptor encephalitis is increasingly recognized worldwide. We compare the clinical features of adults and children with this disorder in Taiwan.Patients admitted to Chang Gung Memorial Hospital and Chang Gung Children's Hospital and those who were referred from other institutions because of unknown encephalitis from 2009 to 2013 were enrolled, and their clinical features were analyzed. Data on cases from a review of the literature were also included in the analysis.Twelve patients (10 females) aged between 7 years and 28 years with anti-N-methyl-D-aspartate receptor encephalitis were identified. Six patients (50%) were18 years old, one of whom was male and three of whom had an underlying tumor. Overall, 91.6% of the patients presented with mood, behavioral, or personality changes; 91.6% developed seizures; 100% had stereotyped movements; 83.3% had autonomic instability; and 66.7% had hypoventilation. Responses to immunotherapy were slow and variable. Overall, 63.6% of the patients had a substantial recovery after immunotherapy or removal of the tumor, and one patient experienced neurological relapses. There were no significant differences in clinical manifestations between children and adults.Anti-N-methyl-D-aspartate receptor encephalitis is increasingly recognized in Taiwan. It is characterized by its clinical features, predominantly affects females with and/or without an ovarian tumor, and it is a potentially treatable disorder. It is important for neurologists to be familiar with the clinical presentations of the disease in children and young adults.

Brain injury after resuscitation is associated with high morbidity and mortality in children. Therapeutic hypothermia has theoretical benefits on brain preservation. It has been shown to be effective in improving neurological outcomes after adult ventricular arrhythmia-induced cardiac arrest and neonatal asphyxia. However, there have only been a few reports about therapeutic hypothermia after pediatric resuscitation. We conducted this retrospective cohort study in a tertiary pediatric intensive care unit between January 2010 and June 2012. All children from 2 months to 18 years of age with resuscitation and a history of at least 3 minutes of chest compressions with survival for 12 hours or more after return of circulation were eligible. Forty-three patients met the eligibility criteria for the study. Forty-two patients (97.6%) were asphyxial in etiology; 29 (67.4%) of them occurred out-of-hospital. Excluding one child with cardiac etiology, the overall survival rate to hospital discharge was 57.1%. Fourteen (33.3%) patients received hypothermia therapy and were cooled to 33°C for 72 hours. The survival rate was higher in the therapeutic hypothermia group (11/14, 78.6%) than in the normothermia group (13/28, 46.4%). In conclusion, therapeutic hypothermia was associated with increased survival rate after pediatric resuscitation.

Encephalitis may present with seizures or status epilepticus, and the etiology is usually presumed. Specific antibodies to ion channels, receptors, and other synaptic proteins were identified during the past decade. However, only a few studies investigated antiglutamic acid decarboxylase antibodies and antibodies to cell membrane ion channels or surface antigens in pediatric encephalitis and status epilepticus. We examined antibodies to glutamic acid decarboxylase and cell membrane ion channels or surface antigens in acute-phase serum from 17 children with encephalitis and status epilepticus. Antiglutamic acid decarboxylase antibody titers were compared with those of control children manifesting therapy-resistant epilepsy. Antiglutamic acid decarboxylase antibody titers were significantly higher in those with encephalitis and status epilepticus than in those with therapy-resistant epilepsy. No patient demonstrated antibodies to cell membrane ion channels or surface antigens. Six children exhibited positive antiglutamic acid decarboxylase antibodies. One child died, three manifested postencephalitic epilepsy with neurologic deficits, and two recovered well. Higher antiglutamic acid decarboxylase antibody titers were evident in the children with encephalitis and status epilepticus. Clinicians should be aware of the potential role of antiglutamic acid decarboxylase antibodies in children with encephalitis and status epilepticus.

Central diabetes insipidus occurs in patients with overwhelming central nervous system injuries, and may be associated with brain death. The clinical picture of children with acquired central diabetes insipidus after acute brain insult is seldom reported. We retrospectively reviewed cases dating from January 2000-February 2008 at a tertiary pediatric intensive care unit. Fifty-four patients (28 girls, 26 boys), aged 3 months to 18 years, were enrolled. Etiologies included severe central nervous system infection (35.2%), hypoxic-ischemic events (31.5%), head injury (18.5%), and vascular lesions (14.8%). In 39 (72.2%) patients, diabetes insipidus was diagnosed during the first 2 days after acute central nervous system injury, and 40 (74.0%) developed maximum serum sodium concentrations of >160 mEq/L. In 16, sequential cerebral salt wasting syndrome developed after their initial diabetes insipidus presentation. Overall mortality at 2 months after admission was 77.8%. Our results demonstrate that patients who develop central diabetes insipidus after acute central nervous system injury manifest high mortality. Development of central diabetes insipidus within the first 2 days and a maximum plasma sodium >160 mEq/L were significant predictors of outcomes.

Neurogenic stunned myocardium is characterized by reversible left ventricular dysfunction, which occurs after severe central nervous system injury in patients without coronary artery disease. It has been described in association with subarachnoid hemorrhage, Guillain-Barre syndrome, and metastatic brain tumors in adults, but has been rarely reported in association with acute myelitis. Described here is a novel case of acute myelitis in a child associated with neurogenic stunned myocardium. This case demonstrates that patients with acute myelitis may harbor a risk of sympathetic dysregulation, leading to neurogenic myocardial dysfunction.

Convulsive status epilepticus is a medical emergency with significantly associated mortality and morbidity. The demographic data and outcomes of convulsive status epilepticus in children were collected for descriptive analysis. We retrospectively reviewed cases of convulsive status epilepticus in the Pediatric Intensive Care Unit of Chang Gung Children's Hospital between 1999 and 2006. We enrolled 141 patients with 198 episodes of convulsive status epilepticus, aged 2 months to 18 years: 24.8% of first episodes developed convulsive status epilepticus, with a duration of over 60 minutes. First episodes of convulsive status epilepticus were most often evidenced in febrile status during acute central nerve system infections (48.2%), and in nonfebrile status during acute noncentral nerve system illness in previously epileptic children (28.4%). Before their first episode, 63.8% of children were neurologically healthy, and 12.2% exhibited a prolonged febrile seizure. The most common etiology of mortality was acute central nervous system infection. The immediate mortality rate was 7.1%. Convulsive status epilepticus in childhood is more common, with a different range of causes and a lower risk of death, than convulsive status epilepticus in adults. Acute central nervous system infections appear to be markers for morbidity and mortality.

Central diabetes insipidus, a common consequence of acute central nervous system injury, causes hypernatremia; cerebral salt wasting syndrome can cause hyponatremia. The two conditions occurring simultaneous are rarely described in pediatric patients. Pediatric cases of combined diabetes insipidus and cerebral salt wasting after acute central nervous system injury between January 2000 and December 2007 were retrospectively reviewed, and clinical characteristics were systemically assessed. Sixteen patients, aged 3 months to 18 years, met study criteria: 11 girls and 5 boys. The most common etiologies were severe central nervous system infection (n = 7, 44%) and hypoxic-ischemic event (n = 4, 25%). In 15 patients, diabetes insipidus was diagnosed during the first 3 days after acute central nervous system injury. Onset of cerebral salt wasting syndrome occurred 2-8 days after the onset of diabetes insipidus. In terms of outcome, 13 patients died (81%) and 3 survived under vegetative status (19%). Central diabetes insipidus and cerebral salt wasting syndrome may occur after acute central nervous system injury. A combination of both may impede accurate diagnosis. Proper differential diagnoses are critical, because the treatment strategy for each entity is different.

Posterior circulation infarction is uncommon in children. Vertebral artery dissection is an unusual cause of posterior circulation infarction in children. We report on a 12-year-old boy with spontaneous left-extracranial vertebral artery dissection associated with isolated ipsilateral superior cerebellar artery territory infarction, diagnosed clinically and by brain computed tomography, magnetic resonance imaging, and magnetic resonance angiography. Cerebral angiography demonstrated a flame-like occlusion of the left vertebral artery at level C 2 -C 3 , and indicated that artery-to-artery embolus may be a mechanism of superior cerebellar artery territory infarction. We emphasize that vertebral artery dissection should be considered in a child with acute signs of posterior circulation ischemia.