Neurological Etiologies and Pathophysiology of Cyclic Vomiting Syndrome.

Cyclic vomiting syndrome is an idiopathic chronic periodic disorder of childhood which may persist into the adult years. Although cyclic vomiting syndrome is considered a central nervous system disorder, it is often managed by a pediatric gastroenterologist. The practitioner should not assume a gastrointestinal or non-neurological cause of symptoms especially if there are coexisting neurological symptoms and signs or if vomiting does not bring relief; this suggests a possible central nervous system cause, which may necessitate a pediatric neurology consultation. Examples of central nervous system causes of cyclic vomiting syndrome that can have subjective and objective neurological findings include abdominal migraine, certain types of epilepsy, structural lesions (tumors, Chiari malformation, demyelinating disease), mitochondrial disease, autonomic disorders, fatty acid/organic acid disorders, urea cycle defects, and cannabinoid hyperemesis syndrome. Improved familiarity with cyclic vomiting syndrome and its mimics may improve the time to appropriate diagnosis and may reduce morbidity related to cyclic vomiting syndrome.

Young Patients With Diabetes Have Decreased Cerebrovascular Reactivity Under Hypercapneic Conditions.

OBJECTIVE: Adult diabetic patients have an abnormal cerebrovascular response to hypercapnia, but there are few studies focused on diabetes mellitus type 1 and cerebral blood flow in pediatric or adolescent patients. We hypothesize that young patients with diabetes exhibit a different response to hypercapnia than normal control counterparts. METHODS: Using transcranial Doppler techniques, we compared young diabetic patients with healthy controls by measuring cerebral blood velocity before and during carbon dioxide challenge. RESULTS: Subjects with diabetes had decreased cerebral blood velocity reactivity when compared with the control group (P = 0.005). CONCLUSION: Our results suggest cerebrovascular dysfunction in diabetic patients beginning at an early age. The possibility of long-term implications for cerebrovascular disease demonstrates the need for further studies in the pediatric and adolescent diabetic population to better understand this prevalent condition.

Executive function and cerebrovascular reactivity in pediatric hypertension.

Primary hypertension is associated with decreased performance on neurocognitive testing and a blunted cerebrovascular reactivity to hypercapnia. Parents of 14 children with hypertension and prehypertension completed the Behavior Rating Inventory of Executive Functions. Children underwent 24-hour ambulatory blood pressure monitoring and transcranial Doppler with reactivity measurement using time-averaged maximum mean velocity and end-tidal carbon dioxide during hypercapnia-rebreathing test. Comparing the reactivity slope for the patients to historical controls showed a statistically significant difference (t = -5.19, df = 13, P < .001), with lower slopes. Pearson correlations of the Behavior Rating Inventory of Executive Functions scores with the reactivity slopes showed a statistically significant inverse relationship with Behavioral Regulation Index (r = -.60, P = .02), Metacognition Index (r = -.40, P = .05), and the Global Executive Component (r = -.53, P = .05). Children with hypertension have decreased executive function, and this correlates to low transcranial Doppler-reactivity slopes, suggesting that the brain is a target organ in hypertensive children.

Brain hypoperfusion in a girl with systemic lupus erythematosus.

We describe an adolescent girl with systemic lupus erythematosus, presenting with severe cardiovascular autonomic dysfunction and incapacitating orthostatic hypotension to a degree not previously reported. Further evaluation of cerebral blood flow velocity, using transcranial Doppler testing, demonstrated an abnormal hypercapnic cerebrovascular response. Both the orthostatic hypotension and the abnormal cerebrovascular hypercapnic response improved with intensive medical treatment of her systemic lupus erythematosus. Additional studies are necessary to elucidate the pathogenesis of these cerebrovascular and autonomic abnormalities, especially considering the potential consequences they may exert on cerebral perfusion, which may be subtle, underrecognized, and capable of affecting cognition.

Hypertension impairs vascular reactivity in the pediatric brain.

BACKGROUND AND PURPOSE: Chronic hypertension impairs cerebrovascular regulation in adults, but its effects on the pediatric population are unknown. The objective of this study was to investigate cerebrovascular abnormalities in hypertensive children and adolescents. METHODS: Sixty-four children and adolescents aged 7 to 20 years underwent transcranial Doppler examinations of the middle cerebral artery at the time of rebreathing CO2. Time-averaged maximum mean cerebral blood flow velocity and end-tidal CO2 were used to quantify cerebrovascular reactivity during hypercapnia. Patients were clinically categorized as hypertensive, prehypertensive, or white coat hypertensive based on 24-hour ambulatory blood pressure measurements. Their reactivities were compared with 9 normotensive control subjects and evaluated against baseline mean blood pressure z-scores and loads. RESULTS: Untreated hypertensive children had significantly lower hypercapnic reactivity than normotensive children (2.556 +/- 1.832 cm/s x mm Hg versus 4.256 +/- 1.334 cm/s x mm Hg, P < 0.05). Baseline mean diastolic blood pressure z-scores (r = -0.331, P = 0.037) and diastolic blood pressure loads (r = -0.351, P = 0.026) were inversely related to reactivity. CONCLUSIONS: Untreated hypertensive children and adolescents have blunted reactivity to hypercapnia, indicating deranged vasodilatory reactivity. The inverse relationship between diastolic blood pressure indices and reactivity suggests that diastolic blood pressure may be a better predictor of cerebral end organ damage than systolic blood pressure.

The effects of hypertension on the paediatric brain: a justifiable concern.

The prevalence of hypertension in children is increasing but its neurological effects are under-recognised. Here, we describe acute and chronic effects of childhood hypertension on the nervous system. Acute neurological involvement ranges from posterior reversible encephalopathy syndrome to, possibly, infarction and haemorrhage. Children with chronic hypertension are likely to have learning disabilities and deficiencies in executive function, which are potentially reversible with antihypertensive treatment. These cognitive defects may be secondary to abnormal regulation of cerebral blood flow. Raised blood pressure in childhood could also contribute to the early development of atherosclerosis, which can have both short-term and long-term adverse effects on vasculature. Clinical studies are needed to better define the full clinical range of paediatric hypertension on a child's nervous system. Furthermore, accurate biomarkers to define cognitive abnormalities and cerebral involvement need to be identified.