Patología del líquido cefalorraquídeo. Hidrocefalia, pseudotumor cerebral y síndrome de presión baja

  1. Pascual, F. Higes
  2. Monteiro, G. Carvalho
  3. Margarit, B. Povedano
  4. Izquierdo, A. Yusta
Revista:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Año de publicación: 2019

Título del ejemplar: Enfermedades del sistema nervioso (VIII) Enfermedades cerebelosas, hidrocefalia, enfermedades metabólicas

Serie: 12

Número: 77

Páginas: 4537-4549

Tipo: Artículo

DOI: HTTPS://DOI.ORG/10.1016/J.MED.2019.05.002 DIALNET GOOGLE SCHOLAR

Otras publicaciones en: Medicine: Programa de Formación Médica Continuada Acreditado

Resumen

Resumen La hidrocefalia es una dilatación patológica de los ventrículos cerebrales por un incremento del volumen del líquido cefalorraquídeo (LCR). Se produce generalmente por uno de dos mecanismos básicos fisiopatológicos: defecto de circulación o de reabsorción del LCR, dando lugar a los dos principales tipos de hidrocefalia: comunicante y no comunicante. Se puede producir tanto en el niño como en el adulto, predominando en el niño las causas genéticas y la patología perinatal. Las causas adquiridas se pueden producir en cualquier rango de edad. La hidrocefalia normotensiva o hidrocefalia crónica del adulto es una forma de hidrocefalia comunicante, en la cual hay un aumento patológico del volumen de los ventrículos cerebrales con presión de apertura del LCR normal. Se caracteriza por una tríada de síntomas: trastorno de la marcha, deterioro cognitivo e incontinencia de esfínteres y es potencialmente reversible mediante un shunt de derivación. La hipertensión intracraneal (HPTIC) benigna, llamada también pseudotumor cerebri, se define por unos criterios clínicos que se refieren a un cuadro de HPTIC con elevación de la presión de apertura del LCR en punción lumbar (PL), con una composición normal del mismo y descartadas otras causas de HPTIC con las pruebas oportunas. Si bien es considerado un cuadro benigno, hay riesgo de pérdida de visión permanente. El síndrome de hipotensión intracraneal tiene una etiología y presentación clínica variable que se produce, en la mayoría de casos, por fuga del LCR a través del saco tecal de forma espontánea o secundaria a PL, cirugía, traumatismos o sobredrenaje de un shunt de derivación. Cefalea ortostática, disminución de la presión de apertura del LCR y captación meníngea en resonancia magnética son las manifestaciones más características. Hydrocephalus is the pathological enlargement of the brain ventricles caused by accumulation of cerebrospinal fluid (CSF). Two basic pathophysiological mechanisms are involved: impairment of CSF circulation or resorption; causing the two main types of hydrocephalus: communicating and noncommunicating hydrocephalus. Appears both in childhood and adulthood. Genetic causes and perinatal pathology dominate in children. Acquired causes are involved in any age range. Normal pressure hydrocephalus or chronic adult hydrocephalus is a type of communicating hydrocephalus, in which ventricles of the brain enlarge with normal CSF opening pressure. Clinically is characterized by the triad of abnormal gait, sphincter incontinence and cognitive impairment. In most cases, the placement of a cerebrospinal fluid shunt revert it. Idiopathic intracranial hypertension (IIH) or pseudotumor cerebri, is clinically characterized by an increased CSF opening pressure in lumbar puncture (LP), the composition of the cerebrospinal fluid is normal, and no other cause of intracranial hypertension are identified. Although is considered benign, permanent blindness can occur. Intracranial hypotension syndrome has a diverse etiology and variable clinical presentation; usually, spontaneous or secondary (LP, surgery, trauma or shunt) CSF leaks through thecal sac causes it. The most characteristic symptoms are orthostatic headache, lower CSF opening pressure and meningeal contrast enhancement.

Referencias bibliográficas

  • Banta JT, Farris BK. Pseudotumor cerebri and optic nerve sheath decompression. Arch Ophthalmology. 2000;107:1907-12.
  • Bech RA, Juhler M, Waldemar G, Klinken L, Gjerris F. Frontal brain and leptomeningeal biopsy specimens correlated with cerebrospinal fluir out flow resistance and B-wave activity in patients suspectec of normal pressure hydrocephalus. Neurosurgery. 1997;40:497-502.
  • Beck J, Ulrich CT, Fung C, Fichtner J, Seidel K, Fiechter M. Diskogenic microspurs as a major cause of intractable spontaneous intracranial hypotension. Neurology. 2016;87:1220-6.
  • Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, Valade D. Early epidural blood patch in spontaneous intracranial hypotension. Neurology. 2004;63:1950-1.
  • Binder DK, Dillon WP, Fishman RA, Schmidt MH. Intratecal saline infusion in the treatement of obtundation associated with spontaneous intracranial hypotension: technical case report. Neurosurgery. 2002;51:830-6
  • Biousse V, Bruce BB, Newman NJ. Update on the pathophysiology and management of idiophatic intracranial hypertension. J Neurol Neurosurg Psychiatry. 2012;83:488-94.
  • Bradley WG Jr, Whittemore AR, Watanabe AS, Davis SJ, Teresi LM, Homyak M. Association of deep qhite matter infarction wuith chronic communicating hydrocephalus: implications regarding the possible origin or normal pressure hydrocephalus. AJNT Am J Neuroradiol. 1991;12:31-9.
  • Brodsky MC, Vaphiades M. Magnetic resonance imaging in pseudotumor cerebri. Ophtalmology. 1998;105:1686-93.
  • Bruce BB, Dedar S, Van Stavern GP, Monaghan D, Acierno MD, Braswell RA. Idiophatic intracranial hypertension in men. Neurology. 2009;72:304-9.
  • Celebisoy N, Gökçay F, Sirin H, Akyürekii O. Treatment of idiopathic intracranial hypertension topiramate vs acetazolamide an open label study. Acta Neurol Scand. 2007;116:322-7.
  • Chen IH, Huang Cl, Liu HC, Chen KK. Effectiveness of shun-ting in patients with normal pressure hydrocephalus predicted by temporary controlled resistance continuous lumbar drainage. A pilot study. J Neurol Neurosurg Psychiatry. 1994;57:1430-2.
  • Espay AJ, Da Prat GA, Dwivedi AK, Rodriguez-Porcel F, Vaughan JE, Rosso M. Deconstructing normal pressure hydrocephalus: Ventriculomegaly as aearly sign of neurodegeneration. Ann Neurol. 2017;82:503-13.
  • Evans RW, Boes CJ. Spontaneous low cerebrospinal fluid pressure syndrome can mimic primary cough headache. Headache. 2005;45:374-7.
  • Farb RI, Vanek I, Scott JN, Mikulis DJ, Willinsky RA, Tomlinson G. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenosus stenosis. Neurology. 2003;60:1418-24.
  • Franzini A, Messina G, Nazzi V, Mea E, Leone M, Chiapparini L. Spontaneous intracranial hypotension sydrome: a novel speculative physiopathological hypothesis a novel patch method in a series of 28 consecutive patients. J Neurosurg. 2010;112:300-6.
  • Friedman DI, Jacobson DM. Diagnostic criteria for idiophatic intracranial hypertension. Neurology. 2002;59:1492-5.
  • Graff-Radford NR, Godersky JC. Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus. Neurology. 1989;39:1596-600
  • Hoffmann J, Goadsby PJ. Update on intracranial hypertension and hypotension. Curr Opin Neurol. 2013;26:240-7.
  • Jaraj D, Rabiei K, Marlow T, Jensen C, Skoog I, Wikkelsø C. Prevalence of idiopathic normal pressure hydrocephalus. Neurology. 2014;82:1449-54.
  • Kumar N, Diehn FE, Carr CM, Verdoorn JT, Garza I, Luetmer PH. Spinal CSF venous fistula: A treatable etiology for CSF leaks in craniospinal hypovolemia. Neurology. 2016;86:2310-2.
  • Kuriyama N, Tokuda T, Miyamoto J, Takayasu N, Kondo M, Nakagawa M. Retrograde jugular flow associated with idiophatic normal pressure hydrocephalus. Ann Neurol. 2008;64:217-21.
  • Lay CL, Campbell JK, Mokri B. Low cerebrospinal fluid pressure headache. Boston: Headache Butterwotrh-Heinemann; 1997. p. 355.
  • Leep Hunderfund AN, Mokri B. Second half of the day headache as a manifestation of spontaneous CSF leak. J Neurol. 2012;259:306-10.
  • McGirt MJ, Woodworth G, Thomas G, Miller N, Williams M, Rigamonti D. Cerebrospinal fluir shunt placement for pseudotumor cerebri associated intractable headache: predictors of treatment response and an analysis of long term outcomes. J Neurosurg. 2004;101:627-32.
  • Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolium enhancement. Mayo Clin Proc. 1997;72:400-13.
  • Mokri B, Hunter SF, Atkinson JL, Piepgras DG. Orthostatic headaches caused by CSF leak but normal CSF pressures. Neurology. 1998;51:786-90.
  • Mokri B, Aksamit AJ, Atkinson JL. Paradoxical postural headaches in cerebrospinal fluid leaks. Cephalalgia. 2004;24:883-7.
  • Pérez MA, Bialer OY, Bruce BB, Newman NJ, Biousse V. Primari spontaneous cerebrospinal fluid leaks and idiophatic intracranial hypertension. J Neuroophtalmol. 2013;33:330-7.
  • Plotnik JL, Kosmosrsky GS. Operative complications of optic nerve sheath decompression. Ophthalmology. 1993;100:683-90.
  • Puffer RC, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J. Neurointerv Surg. 2013;5:483-6.
  • Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI. Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study. Eur J Hum Genet. 2013;21:386-90.
  • Santillan A, Aamodt W, Bhavarahju-Sanka R. Pearls & Oysters: Spontaneous intracranial hypotension and posterior reversible encephalopathy syndrome. Neurology. 2016;86:e55-7.
  • Schievink WI. Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks. Curr Neurol Neurosci Rep. 2013;13:358.
  • Schievink WI, Maya MM, Moser FM. Treatment of spontaneous intracranial hypotension with percutaneous placement of a fibrin sealant Re-port of four cases. J Neurosug. 2004;100:1098-100.
  • Schievink WI, Maya MM. Spinal meningeal diverticula, spontaneous intracranial hypotension and superficial siderosis. Neurology. 2017;88:916-17.
  • Schievink WI, Maya MM, Chow W, Louy C. Reversible cerebral vasoconstriction in spontaneous intracranial hypotension. Headache. 2007;47:284-7.
  • Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology. 2001;57:1921-3.
  • Sinclair AJ, Burdon MA, Nigtingale PG, Ball AK, Good P, Matthews TD. Low energy diet and intracranial pressure in women with idiopathic intracranial hypertension prospective cohort study. BMJ. 2010;341:c2701.
  • Taktakishvili O, Shah VA, Shabaz R, Lee AG. Recurrent idiophatic intracranial hypertension. Ophtalmology. 2008;115:221.
  • Toma AK, PapadopoulosMC, Stapleton S, Kitchen ND, Watkins LD. Systematic review of the outcome of shunt surgery in idiopathic normal pressure hydrocephalus. Acta Neurochir (Wien). 2013;155:1977-80
  • Wall M, George D. Idiophatic intracranial hypertension. A prospective study of 50 patients. Brain. 1991;114(Pt 1A):155-80.
  • Wall M, Kupersmith MJ, Kiebutx KD, Corbett JJ, Feldon SE, Friedman DI. The idiophatic intracranial hypertension treatment trial clinical profile at baselina. JAMA Neurol. 2014;71:693-701.
  • Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA. Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD. Neurology. 2011;76:1377-82.