Idiopathic intracranial hypertension (pseudotumor cerebri) in pediatric patients

Lange

et al. 2002

Arq. Neuro-Psiquiatr.

-Chronic headaches, associated with papilledema and pulsatile tinnitus without any neuroradiologic, cytobiochemical or cerebrospinal fluid abnormalities are suggestive of idiopathic intracranial hypertension (IIH). However the absence of the papilledema does not rule out this diagnosis. The reason why some patients do not develop papilledema in IIH is ignored, however there are some hypotheses concerning the structure of the optical nerve. In this study we described two female patients that presented diagnosis of IIH with papilledema, with subsequent resolution of papilledema without the due resolution of intracranial hypertension. The long-term behavior of the optic nerve (ON) facing an increased intracranial pressure was evaluated through repeated measurements of the intracranial pressure. We concluded that the ON submitted to high intracranial pressure for a certain lenght of time can adapt itself with subsequent disappearance of the papilledema. The presence or not of papilledema in IIH can be related to the period in which the diagnosis is accomplished.KEY WORDS: headache, intracranial pressure, papilledema, pseudotumor cerebri.Avaliação do comportamento do papiledema na hipertensão intracraniana idiopática: a propósito de Avaliação do comportamento do papiledema na hipertensão intracraniana idiopática: a propósito de Avaliação do comportamento do papiledema na hipertensão intracraniana idiopática: a propósito de Avaliação do comportamento do papiledema na hipertensão intracraniana idiopática: a propósito de Avaliação do comportamento do papiledema na hipertensão intracraniana idiopática: a propósito de dois casos dois casos dois casos dois casos dois casos RESUMO -Cefaléias com características crônica, diária, acompanhadas de edema de papila e tinitus pulsátil, sem nenhum achado neuroradiológico ou citobioquímico no líquor, são altamente sugestivas de hipertensão intracraniana idiopática (HII). Entretanto a ausência do papiledema não invalida o seu diagnóstico. A razão pela qual alguns pacientes não desenvolvem papiledema na HII é desconhecida, porém algumas hipóteses relacionadas com propriedades intrínsecas da bainha do nervo óptico têm sido propostas. Neste estudo relatamos dois pacientes do sexo feminino que apresentaram diagnóstico de HII com papiledema, evoluindo para resolução do papiledema sem a devida resolução da HII. O comportamento do nervo óptico (NO) frente ao aumento da pressão intracraniana foi avaliado neste estudo a partir de um monitoramento intermitente criterioso da pressão intracraniana. Concluímos que o NO submetido a um período de hipertensão intracraniana pode adaptar-se a este ambiente promovendo uma resolução do papiledema para normalidade papilar. A presença ou ausência de papiledema na HII pode estar relacionada ao período no qual seu diagnóstico é realizado. PALAVRAS-CHAVE: cefaléia, pressão intracraniana, papiledema, pseudotumor cerebral.

Section: Discussionmentioning

confidence: 99%

Long-term evolution of papilledema in idiopathic intracranial hypertension: observations concerning two cases

Lange

et al. 2002

Arq. Neuro-Psiquiatr.

“…Both had pupillary light/near disassociation, one had limited upward gaze and the other had a bilateral intranuclear ophthalmoplegia. 5 Our patient had no visual symptoms or signs at presentation or during initial treatment. It was only after the acetazolamide had been gradually tapered over 3 months that the symptom of blurred vision was noted and resolved with an increased dosage of the medication.…”

Section: Commentsmentioning

confidence: 99%

Idiopathic Intracranial Hypertension in a Young Child Without Visual Symptoms or Signs

Winner¹,

Bello²

1996

Headache

A 7-year-old boy presented with a 4-week history of daily headache. His parents reported that he was unable to attend school the week prior to presentation. Intermittent nausea without vomiting was reported, but no blurred vision, photophobia, or diplopia were described. There was no history of trauma or recent systemic illness. The physical examination showed mild neck discomfort, no papilledema, and normal cranial nerve, motor and sensory functioning. Both a CT scan of the sinuses and an MRI of the brain were normal. Although the opening pressure was elevated, the cerebrospinal fluid was also normal. In previous accounts idiopathic intracranial hypertension in children, concomitant papilledema, visual symptoms and/or palsy of the sixth cranial nerve are described. This case demonstrates that idiopathic intracranial hypertension in a young child can present as a daily headache without any visual symptoms or signs. Key words: idiopathic intracranial hypertensionAbbreviations: IIH idiopathic intracranial hypertension (Headache 1996;36:574-576) Idiopathic intracranial hypertension (pseudotumor cerebri) (IIH) is traditionally defined as a syndrome that satisfies the following criteria: (1) clinical manifestations of raised intracranial pressure, (2) normal cerebrospinal fluid (CSF) analysis, and (3) the absence of space-occupying lesions with normal or small ventricles on neuroimaging studies of the brain. Either unilateral or bilateral papilledema is assumed to be present. Double vision caused by abducens palsy may be present, but the remainder of the neurological examination should be unremarkable. In a study of children with IIH, Dhiravibulya et al reported papilledema in 96%, blurred vision in 57%, abducens palsy in 48%, and visual impairment in 45%. 1 Here we describe a young child with IIH without any visual symptoms or signs, and the outcome of medical management. CASE HISTORYA 7-year-old boy presented with a 4-week history of daily, bifrontal, progressive headaches. During the first 3 weeks, the headaches had fluctuated from mild to moderate. In the week prior to presentation, the headaches had been severe, and he had been unable to attend school. No reports of blurred vision, photophobia, phonophobia, or diplopia could be elicited either from the parents or child by interview. There was no history of recent trauma or systemic illness. Intermittent nausea without vomiting was reported, however, and the headache had not responded to acetaminophen or ibuprofen.The parents reported that the child began to have headaches at 5 years of age. The headaches were bifrontal, lasting 1 to 2 hours and occurring at 1-to 2-month intervals. There was no associated photophobia or phonophobia, although there was nausea without vomiting. The headaches responded to acetaminophen and/or sleep.The patient's prenatal and natal histories were unremarkable. No exposure to environmental toxins was reported. The patient had a postnatal pyloric stenosis, which was repaired at 6 weeks of age. Otherwise, the past medical history was...

“…4 Recent studies have noted that the clinical profile of pediatric patients with IIH differs from the adult type, suggesting that the precipitating factors may be different in prepubertal children. 5,6 Unlike the adult distribution, in which there is a strong female predominance, 7 IIH in the prepubertal population shows no sex preference. In addition, the outcome in children seems to be better than that in adults, with spontaneous remission more common.…”

mentioning

confidence: 99%

Optic Nerve Sheath Diameter on MR Imaging: Establishment of Norms and Comparison of Pediatric Patients with Idiopathic Intracranial Hypertension with Healthy Controls

Shofty¹,

Ben-Sira²,

Constantini³

et al. 2011

AJNR Am J Neuroradiol