Early Neurorehabilitation for Patients with Normal Pressure Hydrocephalus
Susan R. Borgaro, PhD
Sally Alcott, MD
Division of Neurology, Barrow Neurological Institute, St. Joseph’s Hospital and Medical Center, Phoenix, Arizona
Inpatient neurorehabilitation is often warranted for patients with normal pressure hydrocephalus (NPH), particularly during the earliest stages after shunt surgery. Therapeutic interventions are focused on improving gait disturbance, incontinence, and cognitive impairment within the context of an interdisciplinary treatment setting. Although many of the therapeutic interventions used with the NPH population are effective with other neurological patients, the efficacy of rehabilitation and specific therapeutic interventions used for NPH has not been studied systematically and empirically. Research is needed to document the outcomes of neurorehabilitative interventions for patients with NPH.
Key Words: neurorehabilitation, normal pressure hydrocephalus
The clinical triad of gait disturbance, urinary incontinence, and cognitive impairment associated with normal pressure hydrocephalus (NPH) lends itself to intervention by the rehabilitation team. This multidisciplinary team includes rehabilitation nursing; physical, occupational, and speech therapies; social work or case management; recreational therapy; and neuropsychology. Treatment interventions are aimed at improving physical, cognitive, and emotional disturbances as well as at providing appropriate discharge goals and plans. Psychoeducation for patients and families; information concerning relevant community resources; and supportive assistance to patients, families, and caregivers also are a focus of treatment.
Typically, patients with NPH are referred to the neurorehabilitation unit after undergoing surgical placement of a shunt. Individual therapies are directed at improving gait disturbance, incontinence, and cognitive impairment. This article describes the role of rehabilitation in treating patients with NPH. The need for outcome research on specific rehabilitation interventions with the NPH population is also highlighted.
Gait disturbance is the principle symptom of NPH and often among the earliest to manifest. When gait disturbance precedes mental deterioration, a more favorable outcome is expected than when it does not.[12,14,15] Individuals with NPH may have difficulty initiating gait or transfers. Typically, length of stride and height of steps are reduced. Eventually, a shuffling, broad-based gait interferes with ambulation.
Disturbances in gait are addressed by the physical therapist. The therapy consists of activities aimed at increasing strength and balance and at improving the quality and safety of gait. An assistive device, family training, and review of the home environment may be needed for any adjustments that will maximize the patient’s safety and independence. Many of the therapeutic principles applied to this population are gleaned from geriatric, fall risk, and Parkinson’s literature.
During the early stages of NPH, urinary incontinence is often reported in patients.[3,19] It can also occur during the latter stages. Typically, urinary incontinence and self-care skills are addressed by nursing and the occupational therapist. Initially, incontinence may be related to an individual’s decline in mobility, but it may become multifactorial associated with a decline in cognition, infection, or other medical factors such as prostate disease. Nursing establishes a toileting program, and the family and treatment team help to ensure regular toileting times to promote continence. The occupational therapist evaluates the patient’s need for adaptive equipment to improve independence and to reduce the burden on caregivers.
The cognitive changes associated with NPH vary from day to day and from patient to patient. The neuropsychologist evaluates the patient’s cognitive and emotional status and consults with the rehabilitation staff. Information obtained from the neuropsychological evaluation is used by speech therapists to help focus cognitive interventions and compensatory strategies. Recommendations and compensatory techniques also are provided to families and caregivers.
Cognitive decline may involve disorientation, confusion, apathy, decreased attention span, reduced speed of information processing, and motor slowing while many cognitive functions, judgment, and self-awareness are preserved relatively well. Unlike primary degenerative disorders such as the dementias (e.g., Alzheimer’s disease), if correctly diagnosed, the deteriorating process of NPH may be reversible by surgical placement of a ventricular shunt. If NPH is left to run its course, however, progressive mental debilitation will occur. In its later stages, NPH resembles primary dementias.
During the early stages of NPH, motor slowing, impaired attention, and difficulties in mental tracking are common. Memory difficulties may be apparent in the early stages although such difficulties can be related to confusion and attention deficits rather than to a primary registration deficit or learning disability. In the initial stages, emotional and psychiatric symptoms, such as agitation, anxiety, depression, and paranoidal delusion, may emerge.[20,31] As the condition worsens, the memory system unequivocally becomes affected. Typically, learning and recall of both visual and verbal material are compromised although episodic memory remains intact. Deficits associated with frontal lobe dysfunction, including verbal fluency, cognitive flexibility, and attention, also are common.
Most research on outcomes after NPH has focused on the effectiveness of shunt placement. Surgery substantially improves gait disturbance,[11,21,25] incontinence, and cognitive impairment. [2,5,6,16,18,22,28] For example, Larsson and colleagues reported improvements in length of step and a decrease of arrestments and gait ataxia as long as 1 year after shunt surgery. Urinary incontinence has improved as soon as 1 week after placement of a shunt. Postoperative improvements have been reported in memory, reaction time, visuospatial functions, wakefulness, and initiative.[10,15,16,19,21,25,30,34] Psychiatric symptoms also have improved after surgery. However, deficits associated with frontal lobe dysfunction, such as attention and cognitive flexibility, have improved little after shunting.[4,8,16,17,31,34,35]
Several factors contribute to improvements after surgery. For example, a brief history of the disorder and a known cause are both important prognostic factors.[2,16,22,37] Compared to the idiopathic form, NPH caused by subarachnoid hemorrhage has been associated with better outcomes.[2,16,32] Similarly, 60% to 75% of patients with secondary NPH[9,34,36] showed improvement compared with 10 to 53% of patients with idiopathic NPH.[5-7,34,36] Other investigators have found no prognostic relevance associated with etiology. Thomsen and colleagues reported improved cognitive function in 80% of patients 1 year after surgery when three or more of the following signs were present: known cause, brief history, low cerebrospinal fluid outflow, small sulci, or periventricular hypodensity on computed tomography.
Although several studies have documented improvements in symptoms after surgical placement of a shunt, none of these studies have examined outcomes after rehabilitation. Yet, many patients undergo intensive therapy in a rehabilitation setting after surgery to help improve gait disturbance, incontinence, and cognitive impairment. Many of the therapeutic interventions used with the NPH population are based on treatment known to be effective for patients with different diagnoses but similar functional and cognitive deficits. Whether early rehabilitation interventions can help improve functioning and facilitate recovery after surgical treatment for NPH has yet to be determined.
Inpatient neurorehabilitation is often warranted for patients with NPH during the early stages of recovery from shunt surgery. Overall clinical improvement after surgical placement of a ventricular shunt has been reported.[23,27,29] However, the efficacy of specific therapeutic interventions used during early rehabilitation has not yet been studied systematically and empirically. Research is needed to document the outcomes associated with the therapeutic interventions used by the multidisciplinary rehabilitation team to treat patients with NPH.
- Adams RD, Fisher CM, Hakim S, et al: Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure: A treatable syndrome. N Engl J Med 273:117-126, 1965
- Belloni G, di Rocco C, Focacci C, et al: Surgical indications in normotensive hydrocephalus. A retrospective analysis of the relations of some diagnostic findings to the results of surgical treatment. Acta Neurochir (Wien) 33:1-21, 1976
- Benson D: The hydrocephalic dementia, in Frederiks JAM (ed): Handbook of Clinical Neurology. Amsterdam, The Netherlands: Elsevier Science, 1985, pp 323-333
- Berglund M, Gustafson L, Hagberg B: Amnestic-confabulatory syndrome in hydrocephalic dementia and Korsakoff’s psychosis in alcoholism. Acta Psychiatr Scand 60:323-333, 1979
- Black PM: Idiopathic normal-pressure hydrocephalus. Results of shunting in 62 patients. J Neurosurg 52:371-377, 1980
- Borgesen SE, Gjerris F, Srensen SC: The resistance to cerebrospinal fluid absorption in humans. A method of evaluation by lumbo-ventricular perfusion, with particular reference to normal pressure hydrocephalus. Acta Neurol Scand 57:88-96, 1978
- Bret P, Chazal J, Janny P, et al: Chronic hydrocephalus in adults [French]. Neurochirurgie 36 Suppl 1:1-159, 1990
- Caltagirone C, Gainotti G, Masullo C, et al: Neurophysiological study of normal pressure hydrocephalus. Acta Psychiatr Scand 65:93-100, 1982
- Cardoso ER, Piatek D, Del Bigio MR, et al: Quantification of abnormal intracranial pressure waves and isotope cisternography for diagnosis of occult communicating hydrocephalus. Surg Neurol 31:20-27, 1989
- Chen IH, Huang CI, Liu HC, et al: Effectiveness of shunting in patients with normal pressure hydrocephalus predicted by temporary, controlled-resistance, continuous lumbar drainage: A pilot study. J Neurol Neurosurg Psychiatry 57:1430-1432, 1994
- Dixon GR, Friedman JA, Luetmer PH, et al: Use of cerebrospinal fluid flow rates measured by phase-contrast MR to predict outcome of ventriculoperitoneal shunting for idiopathic normalpressure hydrocephalus. Mayo Clin Proc 77:509-514, 2002
- Fisher CM: The clinical picture in occult hydrocephalus. Clin Neurosurg 24:270-284, 1977
- George AE, Holodny A, Golomb J, et al: The differential diagnosis of Alzheimer’s disease. Cerebral atrophy versus normal pressure hydrocephalus. Neuroimaging Clin N Am 5:19-31, 1995
- Graff-Radford NR, Godersky JC: Normal-pressure hydrocephalus. Onset of gait abnormality before dementia predicts good surgical outcome. Arch Neurol 43:940-942, 1986
- Graff-Radford NR, Godersky JC, Jones MP: Variables predicting surgical outcome in symptomatic hydrocephalus in the elderly. Neurology 39:1601-1614, 1989
- Gustafson L, Hagberg B: Recovery in hydrocephalic dementia after shunt operation. J Neurol Neurosurg Psychiatry 41:940-947, 1978
- Iddon JL, Pickard JD, Cross JJ, et al: Specific patterns of cognitive impairment in patients with idiopathic normal pressure hydrocephalus and Alzheimer’s disease: A pilot study. J Neurol Neurosurg Psychiatry 67:723-732, 1999
- Kaye JA, Grady CL, Haxby JB, et al: Plasticity in the aging brain. Reversibility of anatomic, metabolic, and cognitive deficits in normal pressure hydrocephalus following shunt surgery. Arch Neurol 47:1336-1341, 1990
- Larsson A, Wikkelso C, Bilting M, et al: Clinical parameters in 74 consecutive patients shunt operated for normal pressure hydrocephalus. Acta Neurol Scand 84:475-482, 1991
- Lezak M: Neuropsychological Assessment. New York: Oxford University Press, 1995
- Malm J, Kristensen B, Karlsson T, et al: The predictive value of cerebrospinal fluid dynamic tests in patients with the idiopathic adult hydrocephalus syndrome. Arch Neurol 52:783-789, 1995
- Mathew NT, Meyers JS, Hartmann A, et al: Abnormal cerebrospinal fluid-blood flow dynamics. Implications in diagnosis, treatment, and prognosis in normal pressure hydrocephalus. Arch Neurol 32:657-664, 1975
- Messert B, Wannamaker BB: Reappraisal of the adult occult hydrocephalus syndrome. Neurology 24:224-231, 1974
- Ogden JA: Neuropsychological and psychological sequelae of shunt surgery in young adults with hydrocephalus. J Clin Exp Neuropsychol 8:657-679, 1986
- Raftopoulos C, Deleval J, Chaskis C, et al: Cognitive recovery in idiopathic normal pressure hydrocephalus: A prospective study. Neurosurgery 35:397-405, 1994
- Rice E, Gendelman S: Psychiatric aspects of normal pressure hydrocephalus. JAMA 223: 409-412, 1973
- Salmon JH: Senile and presenile dementia: Ventriculoatrial shunt for symptomatic treatment. Geriatrics 24:67-72, 1969
- Salmon JH: Adult hydrocephalus. Evaluation of shunt therapy in 80 patients. J Neurosurg 37:423-428, 1972
- Salmon JH, Gonen JY, Brown L: Ventriculoatrial shunt for hydrocephalus ex-vacuo psychological and clinical evaluation. Dis Nerv Syst 32:299-307, 1971
- Stambrook M, Cardosa E, Hawryluk G, et al: Neuropsychological changes following the neurosurgical treatment of normal pressure hydrocephalus. Arch Clin Neuropsychol 3:323-330, 1988
- Stambrook M, Gill D, Cardoso E, et al: Communicating (normal-pressure) hydrocephalus, in Parks RW, Zec RF (eds): Neuropsychology of Alzheimer’s Disease and Other Dementias. New York: Oxford University Press, 1993, pp 104-114
- Stein SC, Langfitt TW: Normal-pressure hydrocephalus. Predicting the results of cerebrospinal fluid shunting. J Neurosurg 41:463-470, 1974
- Stolze H, Kuhtz-Buschbeck JP, Drucke H, et al: Comparative analysis of the gait disorder of normal pressure hydrocephalus and Parkinson’s disease. J Neurol Neurosurg Psychiatry 70:289-297, 2001
- Thomsen AM, Borgesen SE, Bruhn P, et al: Prognosis of dementia in normal-pressure hydrocephalus after a shunt operation. Ann Neurol 20:304-310, 1986
- Torkelson RD, Leibrock LG, Gustavson JL, et al: Neurological and neuropsychological effects of cerebral spinal fluid shunting in children with assumed arrested (“normal pressure”) hydrocephalus. J Neurol Neurosurg Psychiatry 48:799-806, 1985
- Vanneste J, Augustijn P, Dirven C, et al: Shunting normal-pressure hydrocephalus: Do the benefits outweigh the risks? A multicenter study and literature review. Neurology 42:54-59, 1992
- Wood JH, Bartlet D, James AE, Jr., et al: Normal-pressure hydrocephalus: Diagnostics and patient selection for shunt surgery. Neurology 24:517-526, 1974