Idiopathic Intracranial Hypertension (IIH) is a rare neurological disease (incidence <2/100,000/year) whose main feature is a high CSF pressure in absence of brain tumors, and mainly affect young obese women (12-20/100,000/year). IIH diagnosis is based on presence of papilloedema and on the CSF pressure measurement: according to different criteria, the lower bound is 250 or 200mmH2O. The clinical presentation is not homogeneous and the most common signs/symptoms are: headache (75% of cases), vertigo (50%), transient visual obscuration (70%), tinnitus (50%), perioptic subarachnoid space distension (80%), empty sella (75%), posterior globe flattening (60%), transverse sinus narrowing (60%). Prognosis is generally positive, although up to 25% of patients may develop long-term permanent visual loss. IIH heterogeneous presentation and rarity make planning of wide RCT difficult. Therefore, observational studies are of utility as the enable to generate preliminary results and research hypothesis that, in turn, enable to identify core-interest variables, hypothesize longitudinal trends and define sample size for prospective studies. In this report I presented the association of relevant signs/symptoms with IIH diagnosis, the impact of obesity and Binge Eating Disorder (BED) and, finally, patients’ disability profile. The first study reports a retrospective analysis of clinical, radiological and neuro-ophtalmological data referred to 115 patients undertaking diagnostic procedures. It was showed that radiological and neuro-ophtalmological signs/symptoms were more relevant than neurological ones to confirm IIH diagnosis and that the presence of 7 or more signs/symptoms was associated to IIH confirmation, which conversely did not happen with 4 or less. The second study moves from the evidence of a strong association between IIH and obesity, and between BED and obesity: obese-BED patients do not respond well to weight loss programs, but no literature exists on BED-IIH association. We included 57 patients, hospitalized for diagnostic purposes: IIH was confirmed in 38, BED in 7, six of whom had IIH (15.8%). Compared to non-obese ones, obese patients (particularly those with BED) were more likely to have IIH (P<.001), intracranial pressure >200mmH2O (P<.001) and optic nerve atrophy (P=.002). If the sub-group of IIH patients is taken into account, those with BED had higher intracranial pressure (340mmH20 vs.280 mmH20; P=.037) than the non-BED counterparts, while BMI levels were comparable. The third study moves from the paucity of studies addressing patients’ quality of life, and the lack of studies addressing disability, in patients with IIH. We enrolled 38 patients which filled in the WHODAS-12 (average score: 23.6), the MIDAS, BDI-II and SF-36. Results show a relevant disability in these patients: WHODAS-12 average score was higher than that observed in many other neurological patients. Moreover, disability associated to IIH was mostly related to headaches frequency and mood level. In conclusion, these studies are of clinical and management importance as they are based on a sample that is highly representative of daily clinical practice, i.e. patients with clinical features that enable to suppose IIH. The information herein reported opens to new perspectives for the clinical and research activities connected to a rare disease such as IIH: in the long-run, the importance of creating a network of centers with expertise in IIH and of a clinical registry; on a short-term, the importance of completing 12-months follow-up of baseline parameters.
L’ipertensione idiopatica intracranica (IIH) è una patologia neurologica rara (incidenza <2/100.000/anno) caratterizzata da un’elevata pressione del liquor in assenza di tumori cerebrali, e colpisce prevalentemente le giovani donne obese (12-20/100.000/anno). La diagnosi è basata sulla presenza del papilledema e sulla misurazione della pressione intracranica: a seconda dei criteri diagnostici, il limite minimo è fissato in 250mmH2O oppure in 200mmH2O. La patologia si presenta in maniera disomogenea ed i segni/sintomi più comuni sono: cefalea (75% dei casi), vertigini (50%), oscuramento visivo transitorio (70%), tinnito (50%), distensione dello spazio periottico subaracnoideo (80%), sella vuota (75%), appiattimento posteriore dei globi oculari (60%), stenosi o riduzione del flusso a livello dei seni venosi intracranici (60%). La prognosi è tendenzialmente buona, ma il 25% dei pazienti può perdere la vista. L’eterogeneità dell’IIH rende difficile la conduzione di ampi RCT. Gli studi osservazionali sono utili poiché generano risultati preliminari ed ipotesi di ricerca che permettano di definire le variabili di interesse, ipotizzarne l’andamento nel tempo e determinare la dimensione campionaria per studi prospettici. In questo elaborato sono stati studiati l’associazione dei segni e sintomi con la conferma della diagnosi di IIH, l’impatto dell’obesità e del Binge Eating Disorder (BED), ed il profilo di disabilità dei pazienti. Il primo studio presenta un’analisi retrospettiva dei dati clinici, radiologici e neuro-oftalmologici di 115 pazienti ricoverati a scopo diagnostico Lo studio ha mostrato che i segni/sintomi neuro-oftalmologici e radiologici sono più sensibili di quelli neurologici per confermare a diagnosi. Inoltre ha mostrato che la diagnosi di IIH viene confermata se coesistono 7 o più segni/sintomi, mentre non viene confermata se ve ne sono 4 o meno. Il secondo studio parte dall’osservazione della forte associazione fra IIH ed obesità e fra BED ed obesità. I pazienti con BED rispondono meno bene degli obesi senza BED ai programmi di calo ponderale: tuttavia, non esiste letteratura rispetto all’associazione fra IIH e BED. Sono stati inclusi 57 pazienti ricoverati per accertamenti diagnostici: la diagnosi di IIH è stata confermata in 38 pazienti, il BED in 7 pazienti, di cui 6 con diagnosi di IIH (15.8%). In confronto ai pazienti non obesi, gli obesi (specie quelli con BED) hanno più spesso la diagnosi di IIH (P<.001), pressione intracranica superiore a 200mmH2O (P<.001) ed atrofia del nervo ottico (P=.002). Considerando il solo gruppo di pazienti con IIH, gli obesi con BED hanno un livello di pressione intracranica superiore a quello dei pazienti senza BED (340mmH20 vs.280 mmH20; P=.037), a parità di BMI. Il terzo studio è motivato dalla scarsità di dati relativi alla valutazione di qualità di vita, ed all’assenza di studi che abbiano valutato la disabilità, nei pazienti con IIH. Sono stati arruolati 38 pazienti, che hanno compilato il WHODAS-12 (punteggio medio 23.6). I pazienti hanno completato l’iter diagnostico e compilato il MIDAS, il BDI-II ed il questionario SF-36. I risultati mostano l’importante disabilità dei pazienti arruolati (il WHODAS-12 ha un punteggio superiore a quello osservato in altri pazienti neurologici), ed il fatto che questa sia principalmente associata alla frequenza delle cefalee ed al tono dell’umore. Questi studi sono di interesse clinico e gestionale poichè sono basati su di un campione fortemente rappresentativo di ciò che viene visto nella pratica quotidiana: i pazienti con caratteristiche suscettive di IIH. I dati qui descritti aprono nuove prospettive per la gestione clinica e di ricerca in una patologia rara come questa: la creazione di un network di centri che si occupino di IIH e di un registro di malattia e, nel breve termine, il completamento del follow-up – almeno a 12 mesi – dei parametri valutati al baseline.
Studi osservazionali sull’Ipertensione Idiopatica Intracranica: un valido strumento per la comprensione della malattia
RAGGI, ALBERTO
2017-02-21
Abstract
Idiopathic Intracranial Hypertension (IIH) is a rare neurological disease (incidence <2/100,000/year) whose main feature is a high CSF pressure in absence of brain tumors, and mainly affect young obese women (12-20/100,000/year). IIH diagnosis is based on presence of papilloedema and on the CSF pressure measurement: according to different criteria, the lower bound is 250 or 200mmH2O. The clinical presentation is not homogeneous and the most common signs/symptoms are: headache (75% of cases), vertigo (50%), transient visual obscuration (70%), tinnitus (50%), perioptic subarachnoid space distension (80%), empty sella (75%), posterior globe flattening (60%), transverse sinus narrowing (60%). Prognosis is generally positive, although up to 25% of patients may develop long-term permanent visual loss. IIH heterogeneous presentation and rarity make planning of wide RCT difficult. Therefore, observational studies are of utility as the enable to generate preliminary results and research hypothesis that, in turn, enable to identify core-interest variables, hypothesize longitudinal trends and define sample size for prospective studies. In this report I presented the association of relevant signs/symptoms with IIH diagnosis, the impact of obesity and Binge Eating Disorder (BED) and, finally, patients’ disability profile. The first study reports a retrospective analysis of clinical, radiological and neuro-ophtalmological data referred to 115 patients undertaking diagnostic procedures. It was showed that radiological and neuro-ophtalmological signs/symptoms were more relevant than neurological ones to confirm IIH diagnosis and that the presence of 7 or more signs/symptoms was associated to IIH confirmation, which conversely did not happen with 4 or less. The second study moves from the evidence of a strong association between IIH and obesity, and between BED and obesity: obese-BED patients do not respond well to weight loss programs, but no literature exists on BED-IIH association. We included 57 patients, hospitalized for diagnostic purposes: IIH was confirmed in 38, BED in 7, six of whom had IIH (15.8%). Compared to non-obese ones, obese patients (particularly those with BED) were more likely to have IIH (P<.001), intracranial pressure >200mmH2O (P<.001) and optic nerve atrophy (P=.002). If the sub-group of IIH patients is taken into account, those with BED had higher intracranial pressure (340mmH20 vs.280 mmH20; P=.037) than the non-BED counterparts, while BMI levels were comparable. The third study moves from the paucity of studies addressing patients’ quality of life, and the lack of studies addressing disability, in patients with IIH. We enrolled 38 patients which filled in the WHODAS-12 (average score: 23.6), the MIDAS, BDI-II and SF-36. Results show a relevant disability in these patients: WHODAS-12 average score was higher than that observed in many other neurological patients. Moreover, disability associated to IIH was mostly related to headaches frequency and mood level. In conclusion, these studies are of clinical and management importance as they are based on a sample that is highly representative of daily clinical practice, i.e. patients with clinical features that enable to suppose IIH. The information herein reported opens to new perspectives for the clinical and research activities connected to a rare disease such as IIH: in the long-run, the importance of creating a network of centers with expertise in IIH and of a clinical registry; on a short-term, the importance of completing 12-months follow-up of baseline parameters.File | Dimensione | Formato | |
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