The Adult Hydrocephalus Program at Brigham and Women’s Hospital evaluates and treats adults with normal pressure hydrocephalus (NPH), obstructive hydrocephalus, communicating hydrocephalus, congenital hydrocephalus, Chiari I malformation, pseudotumor cerebri (idiopathic intracranial hypertension), and other disorders of cerebrospinal fluid (CSF) flow such as arachnoid cyst and syringomyelia. The Program is a world leader in cutting edge research designed to find better methods for diagnosing and treating these disorders.
Our multidisciplinary team of neurosurgeons, neurologists, neuroradiolodists, physical therapists and other professionals are expert in the diagnosis and treatment of normal pressure hydrocephalus and other disorders of CSF flow. Each patient is carefully evaluated, and a personalized treatment plan is then developed using the most advanced diagnostic and treatment methods. Read this recent Boston Globe article to learn more about Normal Pressure Hydrocephalus and the Adult Hydrocephalus Program at Brigham and Women's Hospital.
To schedule an appointment, please call (617) 525-8135 or (617) 732-6600.
Normal Pressure Hydrocephalus
If you or someone you know has developed difficulty walking, incontinence or short term memory loss, the problem may be a disorder called normal pressure hydrocephalus. Normal pressure hydrocephalus is thought to be caused by an accumulation of excess cerebrospinal fluid in the brain. Normal pressure hydrocephalus usually occurs in older adults, and it often remains undiagnosed for years or is misdiagnosed as Alzheimer’s Disease, Parkinson’s Disease or “old age”. Normal pressure hydrocephalus can also develop in patients with a history of brain tumor, cerebral hemorrhage, brain infection or head trauma. Importantly, the symptoms of normal pressure hydrocephalus can be relieved by a simple surgical procedure. Our multidisciplinary team is specially trained to diagnose and treat normal pressure hydrocephalus.
Watch this video showing walking difficulty with Normal Pressure Hydrocephalus.
Neurological examination – A careful examination is performed by neurologists or neurosurgeons who are skilled in the diagnosis and treatment of normal pressure hydrocephalus and other disorders that can cause similar symptoms (such as Alzheimer’s Disease or Parkinson’s Disease). Some patients can have normal pressure hydrocephalus as well as one of these other disorders at the same time. Thus, a careful examination that considers all of these disorders is critical.
Brain imaging – Most (but not all) patients with normal pressure hydrocephalus have evidence of excess cerebrospinal fluid in the brain. Our neuroradiologists at The Adult Hydrocephalus Program are knowledgeable in diagnosing all types of hydrocephalus, including normal pressure hydrocephalus and other causes of dementia that can present with similar symptoms. Brain MRI or CT scans are obtained on all patients as part of the evaluation for adult hydrocephalus.
Physical therapy evaluation- Patients undergo a thorough evaluation by our team of physical therapists, all of whom are specially trained in the assessment of gait disorders. Initial timed assessments of gait and balance are performed prior to CSF drainage, and are then repeated daily while the cerebrospinal fluid is being drained. This provides a clear and objective determination of improvement in symptoms.
Lumbar Cerebrospinal Fluid Drainage- Although normal pressure hydrocephalus can be diagnosed with a large volume spinal tap, some patients require a more prolonged period of spinal drainage before improvement occurs. For this reason, patients are admitted for 2 to 3 days of lumbar drainage after undergoing an extensive gait and cognitive evaluation.
The primary treatment for normal pressure hydrocephalus is placement of a ventricular shunt. The ventricular shunt consists of a small catheter that is passed into the fluid space of the brain where the cerebrospinal fluid is located. The catheter is connected to a valve that can be adjusted non-invasively to regulate the rate at which the cerebrospinal fluid is drained. The ventricular catheter and valve, in turn, are connected to a catheter that drains the cerebrospinal fluid into the abdomen where it is reabsorbed. The shunt is completely internal.
In select cases, hydrocephalus can be treated using a procedure called endoscopic third ventriculostomy. This procedure involves puncturing a membrane within the ventricular system to create an alternative pathway for CSF flow.
Obstructive hydrocephalus occurs when the normal flow of cerebrospinal fluid within the brain is physically blocked. This leads to an accumulation of cerebrospinal fluid and increased pressure in the head that can cause headaches, problems with vision, nausea and vomiting, loss of bladder control, imbalance, memory loss or other symptoms. Although it is usually diagnosed in childhood, obstructive hydrocephalus can occur in adults. The Adult Hydrocephalus Program specializes in treating obstructive hydrocephalus, including endoscopic third ventriculostomy, ventricular shunts or surgical removal of the obstruction when appropriate.
Illustration © Lynne Larson 1986-2013
Congenital hydrocephalus develops around the time of birth, but it can persist into adulthood. In some cases, however, congenital hydrocephalus may not be diagnosed until adulthood. The Adult Hydrocephalus Program at Brigham and Women’s Hospital is equipped to diagnose and treat all forms of congenital hydrocephalus. In addition, several physicians in the Program are pediatric neurosurgeons who routinely treat children with hydrocephalus at our affiliated institution, Boston Children’s Hospital. Together with neurosurgeons at Brigham and Women’s Hospital who specialize in treating adult hydrocephalus, they form a multidisciplinary team that covers the full range of disorders affecting the flow of cerebrospinal fluid throughout the lifespan.
Communicating hydrocephalus can develop after head trauma, brain hemorrhage, radiation or chemotherapy, infection, or in the context of tumors. In some cases, the etiology of communicating hydrocephalus is not known. The Adult Hydrocephalus Program at Brigham and Women’s Hospital is equipped to diagnose and treat all forms of communicating hydrocephalus, regardless of the cause.
Pseudotumor Cerebri (Idiopathic Intracranial Hypertension)
Pseudotumor cerebri (also known as Idiopathic Intracranial Hypertension) is characterized by headaches or visual loss due to abnormally elevated pressure in the head. Pseudotumor cerebri usually occurs in young or middle-aged women, although men can be affected as well. The headaches can interfere with one’s ability to perform daily activities. If left untreated, this disorder can lead to visual loss or even blindness. Treatments for pseudotumor cerebri include ventricular shunt placement or weight loss where appropriate. At the Adult Hydrocephalus Program, lumbar punctures and careful eye examinations by our neuro-ophthalmologists are used to diagnose pseudotumor cerebri so that it can be treated, thereby preserving vision and relieving the severe headaches associated with this disorder.
Chiari I Malformation
Patients who complain of chronic headaches localized to the back of the head, or headaches that are made worse by coughing, sneezing or physical exertion may have a disorder called Chiari I Malformation. The symptoms of Chiari I Malformation develop when a portion of the brain (the cerebellum) presses on surrounding brain areas and blocks the normal flow of cerebrospinal fluid. Other symptoms of Chiari I Malformation include gait imbalance, swallowing difficulty, problems with hearing, or numbness, tingling, pain or weakness involving the arms or legs. In some cases, this can cause a fluid collection to develop in the spinal cord (a syrinx), and this can disrupt normal spinal cord function.
Chiari I Malformation is most commonly diagnosed by magnetic resonance imaging and a clinical history that is consistent with the disorder.
The symptoms of Chiari I Malformation can be cured by surgery. During the surgical procedure, our highly-skilled neurosurgeons carefully remove bone near the base of the skull to decompress the brain and spinal fluid passageways while leaving the brain itself undisturbed, thereby restoring the flow of cerebrospinal fluid.
Mark D. Johnson, MD, PhD
E. Antonio Chiocca, MD, PhD
Elizabeth Claus, MD, PhD
Alan R. Cohen, MD
Ian Dunn, MD
Alexandra Golby, MD
Joseph R. Madsen, MD
Travis S. Tierney, MD, PhD
Benjamin C. Warf, MD
David Matthew Pilgrim, MD
Allan H. Ropper, MD
Srinivasan Mukundan, Jr., MD, PhD
Sashank Prasad, MD
Susan J. Gordon, R.N.
Grace Chen, PT
Tricia Flynn, PT
Christine Iracheta, PT
Rona Carroll, PhD
Bruce Kristal, PhD
Peter J. Park, PhD
Research at the Adult Hydrocephalus Program
The Adult Hydrocephalus Program is working to advance knowledge related to the causes, diagnosis and treatment of adult hydrocephalus. Staff members are world leaders in hydrocephalus research, and collectively they have published over fifty papers on this topic over the past decade. A list of these articles is presented below. Additional basic science, translational and clinical research studies on adult hydrocephalus are currently underway.
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Hydrocephalus-related publications by the Adult Hydrocephalus Program staff since 2003
- Weaver J, Manjila S, Bahuleyan B, Bangert BA, Cohen AR. Rhombencephalosynapsis: embryopathology and management strategies of associated neurosurgical conditions with a review of the literature. J Neurosurg Pediatr. 2013 Mar;11(3):320-6. PMID: 23331215.
- Ginat DT, Prabhu SP, Madsen JR. Postshunting corpus callosum swelling with depiction on tractography. J Neurosurg Pediatr. 2013 Feb;11(2):178-80. PMID: 23157395.
- Warf BC. Congenital idiopathic hydrocephalus of infancy: the results of treatment by endoscopic third ventriculostomy with or without choroid plexus cauterization and suggestions for how it works. Childs Nerv Syst. 2013 Mar 13. PMID: 23483331.
- Warf BC, Bhai S, Kulkarni AV, Mugamba J. Shunt survival after failed endoscopic treatment of hydrocephalus. J Neurosurg Pediatr. 2012 Dec;10(6):463-70. PMID: 23039837.
- Park EH, Eide PK, Zurakowski D, Madsen JR. Impaired pulsation absorber mechanism in idiopathic normal pressure hydrocephalus: laboratory investigation. J Neurosurg. 2012 Dec;117(6):1189-96. PMID: 23061391.
- Alan N, Manjila S, Minich N, Bass N, Cohen AR, Walsh M, Robinson S . Reduced ventricular shunt rate in very preterm infants with severe intraventricular hemorrhage: an institutional experience. J Neurosurg Pediatr. 2012 Nov;10(5):357-64. PMID: 22938077.
- Schiff SJ, Ranjeva SL, Sauer TD, Warf BC. Rainfall drives hydrocephalus in East Africa. J Neurosurg Pediatr. 2012 Sep;10(3):161-7. PMID: 22768966.
- Warf BC, Tracy S, Mugamba J. Long-term outcome for endoscopic third ventriculostomy alone or in combination with choroid plexus cauterization for congenital aqueductal stenosis in African infants. J Neurosurg Pediatr. 2012 Aug;10(2):108-11. PMID: 22747094.
- Rogers EA, Kimia A, Madsen JR, Nigrovic LE, Neuman MI. Predictors of ventricular shunt infection among children presenting to a pediatric emergency department. Pediatr Emerg Care. 2012 May;28(5):405-9. PMID: 22531186.
- Cohen AR. Endoscopic exploration of the basal cisterns: to boldly go where no one has gone before. World Neurosurg. 2012 May-Jun;77(5-6):646-7. PMID: 22120245.
- Warf BC. Educate One to Save a Few. Educate a Few to Save Many. World Neurosurg. 2012 Apr 19. PMID: 22521369.
- Chowdhry SA, Cohen AR. Intraventricular Neuroendoscopy: Complication Avoidance and Management. World Neurosurg. 2012 Feb 10. PMID: 22381833.
- Lin N, Dunn IF, Glantz M, Allison DL, Jensen R, Johnson MD, Friedlander RM, Kesari S. Benefit of ventriculoperitoneal cerebrospinal fluid shunting and intrathecal chemotherapy in neoplastic meningitis: a retrospective, case-controlled study. J Neurosurg. 2011 Oct;115(4):730-6. PMID: 21721878.
- Warf BC. The Impact of Combined Endoscopic Third Ventriculostomy and Choroid Plexus Cauterization on the Management of Pediatric Hydrocephalus in Developing Countries. World Neurosurg. 2011 Nov 7. PMID: 22120411.
- Warf BC, Alkire BC, Bhai S, Hughes C, Schiff SJ, Vincent JR, Meara JG. Costs and benefits of neurosurgical intervention for infant hydrocephalus in sub-Saharan Africa. J Neurosurg Pediatr. 2011 Nov;8(5):509-21. PMID: 22044378.
- Wagshul ME, Eide PK, Madsen JR. The pulsating brain: A review of experimental and clinical studies of intracranial pulsatility. Fluids Barriers CNS. 2011 Jan 18;8(1):5. doi: 10.1186/2045-8118-8-5. PMID: 21349153.
- Warf BC, Dagi AR, Kaaya BN, Schiff SJ. Five-year survival and outcome of treatment for postinfectious hydrocephalus in Ugandan infants. J Neurosurg Pediatr. 2011 Nov;8(5):502-8. PMID: 22044377.
- Cohen AR. Low-pressure hydrocephalus. J Neurosurg. 2011 Nov;115(5):1029-30. PMID: 21800962.
- Warf BC, Dewan M, Mugamba J. Management of Dandy-Walker complex-associated infant hydrocephalus by combined endoscopic third ventriculostomy and choroid plexus cauterization. J Neurosurg Pediatr. 2011 Oct;8(4):377-83. PMID: 21961544.
- Leinonen V, Menon LG, Carroll RS, Dello Iacono D, Grevet J, Jääskeläinen JE, Black PM. Cerebrospinal fluid biomarkers in idiopathic normal pressure hydrocephalus. Int J Alzheimers Dis. 2011;2011:312526. PMID: 21660204.
- Warf BC. Hydrocephalus associated with neural tube defects: characteristics, management, and outcome in sub-Saharan Africa. Childs Nerv Syst. 2011 Oct;27(10):1589-94. PMID: 21928025.
- Madsen JR, Abazi GS, Fleming L, Proctor M, Grondin R, Magge S, Casey P, Anor T. Evaluation of the ShuntCheck noninvasive thermal technique for shunt flow detection in hydrocephalic patients. Neurosurgery. 2011 Jan;68(1):198-205. PMID: 21099708.
- Warf BC, Campbell JW, Riddle E. Initial experience with combined endoscopic third ventriculostomy and choroid plexus cauterization for post-hemorrhagic hydrocephalus of prematurity: the importance of prepontine cistern status and the predictive value of FIESTA MRI imaging. Childs Nerv Syst. 2011 Jul;27(7):1063-71. PMID: 21556955.
- Warf BC, Wright EJ, Kulkarni AV. Factors affecting survival of infants with myelomeningocele in southeastern Uganda. J Neurosurg Pediatr. 2011 Feb;7(2):127-33. PMID: 21284456.
- Warf BC, Stagno V, Mugamba J. Encephalocele in Uganda: ethnic distinctions in lesion location, endoscopic management of hydrocephalus, and survival in 110 consecutive children. J Neurosurg Pediatr. 2011 Jan;7(1):88-93. PMID: 21194291.
- Li L, Padhi A, Ranjeva SL, Donaldson SC, Warf BC, Mugamba J, Johnson D, Opio Z, Jayarao B, Kapur V, Poss M, Schiff SJ. Association of bacteria with hydrocephalus in Ugandan infants. J Neurosurg Pediatr. 2011 Jan;7(1):73-87. PMID: 21194290.
- Eide PK, Park EH, Madsen JR. Arterial blood pressure vs intracranial pressure in normal pressure hydrocephalus. Acta Neurol Scand. 2010 Oct;122(4):262-9. PMID: 20070274.
- Warf BC; East African Neurosurgical Research Collaboration. Pediatric hydrocephalus in East Africa: prevalence, causes, treatments, and strategies for the future. World Neurosurg. 2010 Apr;73(4):296-300. PMID: 20849782.
- Manjila S, Chim H, Eisele S, Chowdhry SA, Gosain AK, Cohen AR. History of the Kleeblattschädel deformity: origin of concepts and evolution of management in the past 50 years. Neurosurg Focus. 2010 Dec;29(6):E7. PMID: 21121721.
- Cohen AR. Prediction, with restriction. J Neurosurg Pediatr. 2010 Oct;6(4):307-9. PMID: 20887099.
- Kulkarni AV, Warf BC, Drake JM, Mallucci CL, Sgouros S, Constantini S; Canadian Pediatric Neurosurgery Study Group. Surgery for hydrocephalus in sub-Saharan Africa versus developed nations: a risk-adjusted comparison of outcome. Childs Nerv Syst. 2010 Dec;26(12):1711-7. PMID: 20552204.
- Warf BC, Kulkarni AV. Intraoperative assessment of cerebral aqueduct patency and cisternal scarring: impact on success of endoscopic third ventriculostomy in 403 African children. J Neurosurg Pediatr. 2010 Feb;5(2):204-9. PMID: 20121373.
- Park EH, Dombrowski S, Luciano M, Zurakowski D, Madsen JR. Alterations of pulsation absorber characteristics in experimental hydrocephalus. J Neurosurg Pediatr. 2010 Aug;6(2):159-70. PMID: 20672938.
- Warf BC, Mugamba J, Kulkarni AV. Endoscopic third ventriculostomy in the treatment of childhood hydrocephalus in Uganda: report of a scoring system that predicts success. J Neurosurg Pediatr. 2010 Feb;5(2):143-8.. PMID: 20121361.
- Filis AK, Moon K, Cohen AR. Synchronous ventriculoscopic and microsurgical resection of complex craniopharyngiomas. Pediatr Neurosurg. 2009;45(6):434-6. PMID: 20110755.
- Grinberg L, Anor T, Cheever E, Madsen JR, Karniadakis GE. Simulation of the human intracranial arterial tree. Philos Transact A Math Phys Eng Sci. 2009 Jun 13;367(1896):2371-86. PMID: 19414460.
- Warf B, Ondoma S, Kulkarni A, Donnelly R, Ampeire M, Akona J, Kabachelor CR, Mulondo R, Nsubuga BK. Neurocognitive outcome and ventricular volume in children with myelomeningocele treated for hydrocephalus in Uganda. J Neurosurg Pediatr. 2009 Dec;4(6):564-70. PMID: 19951045.
- Filis AK, Moon K, Cohen AR. Symptomatic Subdural Hygroma and Hydrocephalus following Chiari I Decompression. Pediatr Neurosurg. 2009;45(6):425-8. PMID: 20051703.
- Governale LS, Fein N, Logsdon J, Black PM. Techniques and complications of external lumbar drainage for normal pressure hydrocephalus. Neurosurgery. 2008 Oct;63(4 Suppl 2):379-84. PMID: 18981847.
- Warf BC, Campbell JW. Combined endoscopic third ventriculostomy and choroid plexus cauterization as primary treatment of hydrocephalus for infants with myelomeningocele: long-term results of a prospective intent-to-treat study in 115 East African infants. J Neurosurg Pediatr. 2008 Nov;2(5):310-6. PMID: 18976099.
- Cappabianca P, Cinalli G, Gangemi M, Brunori A, Cavallo LM, de Divitiis E, Decq P, Delitala A, Di Rocco F, Frazee J, Godano U, Grotenhuis A, Longatti P, Mascari C, Nishihara T, Oi S, Rekate H, Schroeder HW, Souweidane MM, Spennato P, Tamburrini G, Teo C, Warf B, Zymberg ST. Application of neuroendoscopy to intraventricular lesions. Neurosurgery. 2008 Feb;62 Suppl 2:575-97. PMID: 18596446.
- Zou R, Park EH, Kelly EM, Egnor M, Wagshul ME, Madsen JR. Intracranial pressure waves: characterization of a pulsation absorber with notch filter properties using systems analysis: laboratory investigation. J Neurosurg Pediatr. 2008 Jul;2(1):83-94. PMID: 18590402.
- Miller JP, Fulop SC, Dashti SR, Robinson S, Cohen AR. Rethinking the indications for the ventriculoperitoneal shunt tap. J Neurosurg Pediatr. 2008 Jun;1(6):435-8. PMID: 18518692.
- Williams MA, McAllister JP, Walker ML, Kranz DA, Bergsneider M, Del Bigio MR, Fleming L, Frim DM, Gwinn K, Kestle JR, Luciano MG, Madsen JR, Oster-Granite ML, Spinella G. Priorities for hydrocephalus research: report from a National Institutes of Health-sponsored workshop. J Neurosurg. 2007 Nov;107(5 Suppl):345-57. doi: 10.3171/PED-07/11/345. PMID: 18459897.
- Warf BC. Endoscopic third ventriculostomy and choroid plexus cauterization for pediatric hydrocephalus. Clin Neurosurg. 2007;54:78-82. PMID: 18504900.
- Black PM, Dello Iacono D, Olsen Bailey N, Madsen J. The emerging field of third circulation research. Clin Neurosurg. 2007;54:129-33. PMID: 18504909.
- Madsen JR, Egnor M, Zou R. Cerebrospinal fluid pulsatility and hydrocephalus: the fourth circulation. Clin Neurosurg. 2006;53:48-52. PMID: 17380738.
- Aliev G, Miller JP, Leifer DW, Obrenovich ME, Shenk JC, Smith MA, Lamanna JC, Perry G, Lust DW, Cohen AR. Ultrastructural analysis of a murine model of congenital hydrocephalus produced by overexpression of transforming growth factor-beta1 in the central nervous system. J Submicrosc Cytol Pathol. 2006 Jun-Sep;38(2-3):85-91. PMID: 17784635.
- Keyserling H, Mukundan S Jr. The role of conventional MR and CT in the work-up of dementia patients. Magn Reson Imaging Clin N Am. 2006 May;14(2):169-82. PMID: 16873009.
- Bergsneider M, Egnor MR, Johnston M, Kranz D, Madsen JR, McAllister JP 2nd, Stewart C, Walker ML, Williams MA. What we don't (but should) know about hydrocephalus. J Neurosurg. 2006 Mar;104(3 Suppl):157-9. PMID: 16572631.
- Warf BC. Comparison of endoscopic third ventriculostomy alone and combined with choroid plexus cauterization in infants younger than 1 year of age: a prospective study in 550 African children. J Neurosurg. 2005 Dec;103(6 Suppl):475-81. PMID: 16383244.
- Keyserling H, Mukundan S Jr. The role of conventional MR and CT in the work-up of dementia patients. Neuroimaging Clin N Am. 2005 Nov;15(4):789-802. PMID: 16443491.
- Warf BC. Hydrocephalus in Uganda: the predominance of infectious origin and primary management with endoscopic third ventriculostomy. J Neurosurg. 2005 Jan;102(1 Suppl):1-15. PMID: 16206728.
- Warf BC. Comparison of 1-year outcomes for the Chhabra and Codman-Hakim Micro Precision shunt systems in Uganda: a prospective study in 195 children. J Neurosurg. 2005 May;102(4 Suppl):358-62. PMID: 15926385.
- Kirshtein B, Benifla M, Roy-Shapira A, Merkin V, Melamed I, Cohen Z, Cohen A. Laparoscopically guided distal ventriculoperitoneal shunt placement. Surg Laparosc Endosc Percutan Tech. 2004 Oct;14(5):276-8. PMID: 15492657.
- Scott RM, Madsen JR. Shunt technology: contemporary concepts and prospects. Clin Neurosurg. 2003;50:256-67. PMID: 14677444.
To schedule an appointment, please call (617) 525-8135 or (617) 732-6600.
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This page was last modified on 11/20/2013