eMedicine Specialties > Neurology > Behavioral Neurology and Dementia

Hydrocephalus: Differential Diagnoses & Workup

Author: Alberto J Espay, MD, MSc, Assistant Professor, Department of Neurology, University of Cincinnati
Contributor Information and Disclosures

Updated: Aug 20, 2009

Differential Diagnoses

Brainstem Gliomas
Migraine Headache
Childhood Migraine Variants
Migraine Variants
Craniopharyngioma
Oligodendroglioma
Epidural Hematoma
Pituitary Tumors
Frontal and Temporal Lobe Dementia
Primary CNS Lymphoma
Frontal Lobe Epilepsy
Pseudotumor Cerebri
Frontal Lobe Syndromes
Pseudotumor Cerebri: Pediatric Perspective
Glioblastoma Multiforme
Subdural Empyema
Headache: Pediatric Perspective
Subdural Hematoma
Intracranial Epidural Abscess
Sudden Visual Loss
Intracranial Hemorrhage
Meningioma
Mental Retardation

Other Problems to Be Considered

Brainstem syndromes
Macrocephaly
Hydranencephaly
Chronic subdural hemorrhages
Cerebral atrophy
Cerebral tumors
Periaqueductal glioma
Agenesis of corpus callosum
Septo-optic dysplasia
Neuroimaging of vascular malformations and hematomas of the brain

Workup

Laboratory Studies

  • No specific blood tests are recommended in the workup for hydrocephalus.
  • Genetic testing and counseling might be recommended when X-linked hydrocephalus is suspected.
  • Evaluate cerebrospinal fluid (CSF) in posthemorrhagic and postmeningitic hydrocephalus for protein concentration and to exclude residual infection.

Imaging Studies

  • CT can assess the size of ventricles and other structures.
  • MRI can evaluate for Chiari malformation or cerebellar or periaqueductal tumors. It affords better imaging of the posterior fossa than CT. MRI can differentiate normal pressure hydrocephalus (NPH) from cerebral atrophy although the distinctions may be challenging. Flow voids in the third ventricle and transependymal fluid exudates are helpful. However, numerous suitable patients have a brain pattern suggestive of atrophy and small vessel ischemic disease that may ultimately be NPH.8 Guidelines for imaging studies in suspected NPH have been established.9
  • CT/MRI criteria for acute hydrocephalus include the following:
    • Size of both temporal horns is greater than 2 mm, clearly visible. In the absence of hydrocephalus, the temporal horns should be barely visible.
    • Ratio of the largest width of the frontal horns to maximal biparietal diameter (ie, Evans ratio) is greater than 30% in hydrocephalus.
    • Transependymal exudate is translated on images as periventricular hypoattenuation (CT) or hyperintensity (MRI T2-weighted and fluid-attenuated inversion recovery [FLAIR] sequences).
    • Ballooning of frontal horns of lateral ventricles and third ventricle (ie, "Mickey mouse" ventricles) may indicate aqueductal obstruction.
    • Upward bowing of the corpus callosum on sagittal MRI suggests acute hydrocephalus.
  • CT/MRI criteria for chronic hydrocephalus include the following:
    • Temporal horns may be less prominent than in acute hydrocephalus.
    • Third ventricle may herniate into the sella turcica.
    • Sella turcica may be eroded.
    • Macrocrania (ie, occipitofrontal circumference >98th percentile) may be present.
    • Corpus callosum may be atrophied (best appreciated on sagittal MRI). In this case, parenchymal atrophy and ex-vacuo (rather than true) hydrocephalus from a neurodegenerative disease should be considered.
  • Ultrasonography through the anterior fontanelle in infants is useful for evaluating subependymal and intraventricular hemorrhage and in following infants for possible development of progressive hydrocephalus.
  • Radionuclide cisternography can be done in NPH to evaluate the prognosis with regard to possible shunting. If a late scan (48-72 h) shows persistence of ventricular activity with a ventricular to total intracranial activity (V/T ratio) greater than 32%, the patient is more likely to benefit from shunting.10  Because of its poor sensitivity in predicting shunt response when the V/T ration is less than 32%, this test is no longer commonly used.
  • Skull radiographs may depict erosion of sella turcica, or "beaten copper cranium" (called by some authors "beaten silver cranium"). The latter can also be seen in craniosynostosis.
  • MRI cine is an MRI technique to measure CSF stroke volume (SV) in the cerebral aqueduct. Cine phase-contrast MRI measurements of SV in the cerebral aqueduct does not appear to be useful in predicting response to shunting.11

Other Tests

  • After shunt insertion, confirm correct positioning of installed hardware with a plain radiograph.
  • EEG if seizure occurs

Procedures

  • Lumbar puncture (LP) is a valuable test in evaluating NPH, but should be performed only after CT or MRI of the head. Normal LP opening pressure (OP) should be less then 180 mm H2 O (ie, 18 cm H2 O). Patients with initial OP greater than 100 mm H2 O have a higher rate of response to CSF shunting than those with OPs less than 100 mm H2 O. Improvement of symptoms after a single LP in which 40-50 mL of CSF is withdrawn appears to have some predictive value for success of CSF shunting.
  • Continuous CSF drainage through external lumbar drainage (ELD) is a highly accurate test for predicting the outcome after ventricular shunting in NPH, although false negative results are not uncommon.12
  • Continuous CSF pressure monitoring can help in predicting a patient's response to CSF shunting in NPH. Some patients with normal OP on LP demonstrate pressure peaks of greater than 270 mm H2 O or recurrent B waves. These patients tend to have higher rates of response to shunting than those who do not have these findings. This procedure also could differentiate NPH from atrophy.

Histologic Findings

  • Thinning and stretching of the cortical mantle may be seen as a result of ventricular dilation.
  • In the acute phase, edema of the periventricular white matter is observed. Relatively few neuronal lesions are present. Ventricular ependyma shows cellular flattening and loss of cilia.
  • At a later stage, the edema disappears and is replaced by fibrosis, axonal degeneration, demyelination, focal loss of cerebral cortical neurons, cellular flattening, and further loss of cilia.

More on Hydrocephalus

Overview: Hydrocephalus
Differential Diagnoses & Workup: Hydrocephalus
Treatment & Medication: Hydrocephalus
Follow-up: Hydrocephalus
Multimedia: Hydrocephalus
References
Further Reading
[ CLOSE WINDOW ]

References

  1. Rekate HL. A contemporary definition and classification of hydrocephalus. Semin Pediatr Neurol. Mar 2009;16(1):9-15. [Medline].

  2. Woodworth GF, McGirt MJ, Williams MA, Rigamonti D. Cerebrospinal fluid drainage and dynamics in the diagnosis of normal pressure hydrocephalus. Neurosurgery. May 2009;64(5):919-25; discussion 925-6. [Medline].

  3. Lacy M, Oliveira M, Austria E, Frim MD. Neurocognitive outcome after endoscopic third ventriculocisterostomy in patients with obstructive hydrocephalus. J Int Neuropsychol Soc. May 2009;15(3):394-8. [Medline].

  4. Garne E, Loane M, Addor MC, Boyd PA, Barisic I, Dolk H. Congenital hydrocephalus - prevalence, prenatal diagnosis and outcome of pregnancy in four European regions. Eur J Paediatr Neurol. Apr 30 2009;[Medline].

  5. Partington MD. Congenital hydrocephalus. Neurosurg Clin N Am. Oct 2001;12(4):737-42, ix. [Medline].

  6. Chauvet D, Sichez JP, Boch AL. [Early epidural hematoma after CSF shunt for obstructive hydrocephalus]. Neurochirurgie. Jun 2009;55(3):350-3. [Medline].

  7. Oertel JM, Mondorf Y, Baldauf J, Schroeder HW, Gaab MR. Endoscopic third ventriculostomy for obstructive hydrocephalus due to intracranial hemorrhage with intraventricular extension. J Neurosurg. May 8 2009;[Medline].

  8. Espay AJ, Narayan RK, Duker AP, Barrett ET Jr, de Courten-Myers G. Lower-body parkinsonism: reconsidering the threshold for external lumbar drainage. Nat Clin Pract Neurol. Jan 2008;4(1):50-5. [Medline].

  9. [Guideline] Dormont D, Seidenwurm DJ, Davis PC. Dementia and movement disorders. American College of Radiology (ACR). 2007;[Full Text].

  10. Larsson A, Moonen M, Bergh AC, Lindberg S, Wikkelso C. Predictive value of quantitative cisternography in normal pressure hydrocephalus. Acta Neurol Scand. Apr 1990;81(4):327-32. [Medline].

  11. Kahlon B, Annertz M, Stahlberg F, Rehncrona S. Is aqueductal stroke volume, measured with cine phase-contrast magnetic resonance imaging scans useful in predicting outcome of shunt surgery in suspected normal pressure hydrocephalus?. Neurosurgery. Jan 2007;60(1):124-9; discussion 129-30. [Medline].

  12. Walchenbach R, Geiger E, Thomeer RT, Vanneste JA. The value of temporary external lumbar CSF drainage in predicting the outcome of shunting on normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. Apr 2002;72(4):503-6. [Medline].

  13. Hamilton MG. Treatment of hydrocephalus in adults. Semin Pediatr Neurol. Mar 2009;16(1):34-41. [Medline].

  14. Black PML. Hydrocephalus in adults. In: Youmans JR, ed. Neurological Surgery. Philadelphia: WB Saunders Company; 1996:927-44.

  15. Chang CC, Kuwana N, Noji M, Tanabe Y, Koike Y, Ikegami T. Cerebral blood flow in patients with normal pressure hydrocephalus. Nucl Med Commun. Feb 1999;20(2):167-9. [Medline].

  16. Colak A, Albright AL, Pollack IF. Follow-up of children with shunted hydrocephalus. Pediatr Neurosurg. Oct 1997;27(4):208-10. [Medline].

  17. Czosnyka M, Pickard JD. Monitoring and interpretation of intracranial pressure. J Neurol Neurosurg Psychiatry. Jun 2004;75(6):813-21. [Medline].

  18. Damasceno BP, Carelli EF, Honorato DC, Facure JJ. The predictive value of cerebrospinal fluid tap-test in normal pressure hydrocephalus. Arq Neuropsiquiatr. Jun 1997;55(2):179-85. [Medline].

  19. du Plessis AJ. Posthemorrhagic hydrocephalus and brain injury in the preterm infant: dilemmas in diagnosis and management. Semin Pediatr Neurol. Sep 1998;5(3):161-79. [Medline].

  20. Frim DM, Scott RM, Madsen JR. Surgical management of neonatal hydrocephalus. Neurosurg Clin N Am. Jan 1998;9(1):105-10. [Medline].

  21. Garvey MA, Laureno R. Hydrocephalus: obliterated perimesencephalic cisterns and the danger of sudden death. Can J Neurol Sci. May 1998;25(2):154-8. [Medline].

  22. Goumnerova LC, Frim DM. Treatment of hydrocephalus with third ventriculocisternostomy: outcome and CSF flow patterns. Pediatr Neurosurg. Sep 1997;27(3):149-52. [Medline].

  23. Hoppe-Hirsch E, Laroussinie F, Brunet L, et al. Late outcome of the surgical treatment of hydrocephalus. Childs Nerv Syst. Mar 1998;14(3):97-9. [Medline].

  24. Libenson MH, Kaye EM, Rosman NP, Gilmore HE. Acetazolamide and furosemide for posthemorrhagic hydrocephalus of the newborn. Pediatr Neurol. Mar 1999;20(3):185-91. [Medline].

  25. Mercuri E, Faundez JC, Cowan F, Dubowitz L. Acetazolamide without frusemide in the treatment of post-haemorrhagic hydrocephalus. Acta Paediatr. Dec 1994;83(12):1319-21. [Medline].

  26. Poca MA, Mataro M, Del Mar Matarin M, Arikan F, Junque C, Sahuquillo J. Is the placement of shunts in patients with idiopathic normal-pressure hydrocephalus worth the risk? Results of a study based on continuous monitoring of intracranial pressure. J Neurosurg. May 2004;100(5):855-66. [Medline].

  27. Sainte-Rose C. Hydrocephalus in childhood. In: Youmans JR, ed. Neurological Surgery. Philadelphia: WB Saunders Company; 1996:890-926.

  28. Sansone JM, Iskandar BJ. Endoscopic cerebral aqueductoplasty: a trans-fourth ventricle approach. J Neurosurg. Nov 2005;103(5 Suppl):388-92. [Medline].

  29. Shbeeb MI, O'Duffy JD, Michet CJ Jr, O'Fallon WM, Matteson EL. Evaluation of glucocorticosteroid injection for the treatment of trochanteric bursitis. J Rheumatol. Dec 1996;23(12):2104-6. [Medline].

  30. Tanaka A, Kimura M, Nakayama Y, Yoshinaga S, Tomonaga M. Cerebral blood flow and autoregulation in normal pressure hydrocephalus. Neurosurgery. Jun 1997;40(6):1161-5; discussion 1165-7. [Medline].

  31. Williams MA, Razumovsky AY, Hanley DF. Comparison of Pcsf monitoring and controlled CSF drainage diagnose normal pressure hydrocephalus. Acta Neurochir Suppl. 1998;71:328-30. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

normal pressure hydrocephalus, communicating hydrocephalus, noncommunicating hydrocephalus, obstructive hydrocephalus, arrested hydrocephalus, acute hydrocephalus, gait apraxia, incontinence, dementia, Arnold-Chiari malformation, papilledema, precocious puberty, Dandy–Walker malformation, obesity, delayed onset of puberty, urinary incontinence, Parkinsonism, seizures, toxoplasmosis, Bickers-Adams syndrome, mental retardation, medulloblastoma, astrocytoma, prematurity, achondroplasia, cysticercosis, treatment, diagnosis

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Alberto J Espay, MD, MSc, Assistant Professor, Department of Neurology, University of Cincinnati
Alberto J Espay, MD, MSc is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society
Disclosure: Boehringer-Ingelheim Consulting fee Consulting; Codman Grant/research funds Other; Medtronic Honoraria Speaking and teaching; Medtronic Grant/research funds Other; Allergan Grant/research funds Other; UCB-Schwarz Pharm Honoraria Speaking and teaching; Novartis  Speaking and teaching

Medical Editor

Anthony M Murro, MD, Laboratory Director, Professor, Department of Neurology, Medical College of Georgia
Anthony M Murro, MD is a member of the following medical societies: American Academy of Neurology and American Epilepsy Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Richard J Caselli, MD, Professor, Department of Neurology, Mayo Medical School, Rochester, MN; Chair, Department of Neurology, Mayo Clinic of Scottsdale
Richard J Caselli, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, American Neurological Association, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Howard A Crystal, MD, Professor, Departments of Neurology and Pathology, State University of New York Downstate; Consulting Staff, Department of Neurology, University Hospital and Kings County Hospital Center
Howard A Crystal, MD is a member of the following medical societies: American Academy of Neurology and American Neurological Association
Disclosure: Medivations Honoraria Consulting

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.