Sleep disturbance in pediatric intracranial hypertension.

STUDY OBJECTIVES: There is a well-established association between headache disorders and sleep disturbances in children, but it is unknown whether sleep disturbance plays a role in pediatric intracranial hypertension. The objective of this study was to examine sleep issues related to pediatric intracranial hypertension. METHODS: Patients with intracranial hypertension in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data were collected from the electronic medical record in addition to patient and parent completed questionnaires. Information on sleep behaviors was gathered using the Children's Sleep Habits Questionnaire, and control data were obtained from patient siblings. Statistical analyses were performed using paired t tests or two-sample t tests, as appropriate. RESULTS: Sixty-two pairs of patients and matched sibling controls were compared. We found a statistically significant difference in total sleep disturbance score (control mean, 44.3; patient mean, 48.1; n = 33 pairs, t = -2.2, P = .035), as well as subscale scores of sleep onset delay (control mean, 1.4; patient mean, 1.7; n = 52 pairs, t = -2.53, P = .014), parasomnias (control mean, 8.5; patient mean, 9.5; n = 42 pairs, t = -2.59, P = .013), and sleep-disordered breathing (control mean, 3.1; patient mean, 3.4; n = 44 pairs, t = -2.61, P = .013). No difference was found in bedtime resistance, sleep duration, sleep anxiety, night awakenings, and daytime sleepiness subscales. Furthermore, no difference was found in total sleep disturbance score between patient subsets, including primary vs secondary intracranial hypertension, body mass index, pubertal status, presence of headaches, or intracranial hypertension treatment. CONCLUSIONS: This observational study suggests that pediatric intracranial hypertension is associated with a modest increase in sleep disturbances.

Sleep Disturbance in Pediatric Intracranial Hypertension.

STUDY OBJECTIVES: There is a well-established association between headache disorders and sleep disturbances in children, but it is unknown if sleep disturbance plays a role in pediatric intracranial hypertension. The objective of this study was to examine sleep issues related to pediatric intracranial hypertension. METHODS: Patients with intracranial hypertension who were followed in the Pediatric Intracranial Hypertension Clinic were recruited between July 2017 and September 2018. Demographic data was collected from the electronic medical record in addition to patient and parent completed questionnaires. Information on sleep behaviors was gathered using the Children's Sleep Habits Questionnaire, and control data was obtained from patient siblings. Statistical analyses were performed using paired t-tests or two-sample t-tests, as appropriate. RESULTS: Sixty-two pairs of patients and matched sibling controls were compared. There was a statistically significant difference in total sleep disturbance score (control mean 44.3; patient mean 48.1; n=33 pairs, t=-2.2, p=0.035) as well as subscale scores of sleep onset delay (control mean 1.4; patient mean 1.7; n=52 pairs, t=-2.53, p=0.014), parasomnias (control mean 8.5; patient mean 9.5; n=42 pairs, t=-2.59, p=0.013), and sleep disordered breathing (control mean 3.1; patient mean 3.4; n=44 pairs, t=-2.61, p=0.013). There was no difference found in bedtime resistance, sleep duration, sleep anxiety, night wakings, and daytime sleepiness subscales. Furthermore, there was no difference in total sleep disturbance score between patient subsets including: primary versus secondary intracranial hypertension, body mass index, pubertal status, presence of headaches, or intracranial hypertension treatment. CONCLUSIONS: This observational study suggests that pediatric intracranial hypertension is associated with a modest increase in sleep disturbances.

Neuronal Ceroid Lipofuscinosis and Associated Sleep Abnormalities.

PURPOSE: The aims of this study were to evaluate sleep difficulties in children with neuronal ceroid lipofuscinosis and to determine the association between the sleep difficulties and the onset of seizures and loss of vision. METHOD: We recruited individuals with a confirmed diagnosis of neuronal ceroid lipofuscinosis. We obtained information from the caregiver using the validated Children's Sleep Habits Questionnaire which is a sleep instrument for both behaviorally and medically based problems. Additional information was collected including onset of symptoms, treatment trials, and screen for restless leg syndrome symptoms. RESULTS: In our cohort of 54 individuals, 96.3% had sleep scores consistent with a sleep disturbance. Sleep subscale analysis provided additional insight into the characteristics of the sleep disturbance. Fifty two of the 54 patients had at least one abnormal sleep subscale. The onset of sleep disturbance was associated with the onset of both seizures (ρ = 0.5834, P < 0.0001) and loss of vision (ρ = 0.3840, P = 0.0084). Restless leg syndrome symptoms were reported in 35.2%. CONCLUSION: Children with neuronal ceroid lipofuscinosis have a high burden of sleep disturbances. Using the results of a sleep disturbance screening tool can help to identify the most disturbing symptoms. Targeted treatment of sleep disturbance may improve the quality of life for the patient and family.

Isolated sympathetic failure with autoimmune autonomic ganglionopathy.

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.

Primary diffuse leptomeningeal gliomatosis.

BACKGROUND: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare presentation of a primary central nervous system glial tumor. METHODS: Four case reports of PDLG in young males aged 14-24 years are presented. These reports are discussed in the context of the existing literature. RESULTS: The clinical presentation of 4 new cases of PDLG resembled chronic meningitis with and without polyradiculopathy. Spinal fluid studies are typically nondiagnostic, but characteristically show elevated opening pressure, an elevated protein level, and a relative paucity of cellular reaction. An accurate antemortem diagnosis required contrast-enhanced imaging and meningeal biopsy in all 4 of our cases. Treatment strategies including craniospinal radiation and chemotherapeutic approaches, alone or in combination, have not been proven to alter the course of the disease. Initial responses to temozolomide and radiation treatments in all 4 of our cases were promising, resulting in temporary stabilization of the disease and prolonging life expectancy over what was previously reported in the literature. CONCLUSION: Total neuroaxis contrast-enhanced MRI scanning is required for directing biopsy confirmation and detecting the extent of the disease. More effective therapeutic strategies are needed, but the combination of temozolomide and radiation therapy may slow disease progression.