Summary and Future Perspectives

Educating Medical Personnel

Education of medical personnel is the key point in improving patient care and advancing scientific knowledge–based treatment. This book is part of that effort.

Idiopathic NPH (iNPH) is underdiagnosed, and an estimated 80% of NPH cases remain unrecognized.⁷ Family physicians, neurologists, and psychiatrists are in a key position to differentiate NPH from alternative diagnoses and to refer patients for treatment to a neurosurgeon.^8,9 Of utmost importance is disease awareness.

Results of shunt treatment are better if patients are treated early in the course of the disease.² A cut-off point to achieve the best possible results is at about 1 year after the onset of symptoms. This observation stresses the above-mentioned responsibility of family physicians, neurologists, psychiatrists, and neurosurgeons.

NPH is a chronic condition, meaning that patients are not “cured” after shunt surgery, and their condition will deteriorate again after a certain time period (usually within 3–5 years). Therefore, the potential role for neurologists and family physicians in the long-term management has broadened, not simply by establishing the diagnosis early, but by differentiating NPH from other findings (ventriculomegaly without clinical findings) or diseases (Alzheimer dementia, vascular dementia, Parkinson disease, Huntington disease, spongiform encephalopathy, multiple-system atrophy, corticobasal atrophy) and following up patients after surgical treatment (and possibly more and more with regard to valve adjustments to fit patient needs), all of which have gained increasing importance.¹⁰

Interdisciplinary NPH clinics, staffed by neurologists, neurosurgeons, neuropsychologists, nurses, and social workers, may be one concept for the future to meet the increasing demand.

An excellent example that illustrates this topic is a study published by Harold O. Conn,¹¹ who is a retired faculty member of the Yale University School of Medicine. Interestingly enough, the author of the paper also had NPH. Soon after retirement in 1992, he developed slowness and clumsiness of gait, later urinary incontinence, short-term memory loss, slow responsiveness, and decrease in reaction time and mental sharpness. He was diagnosed by one neurologist with cerebral atrophy and by another colleague with Parkinson disease. He did not respond to pharmaceutical therapy.

The correct diagnosis of NPH was finally made in 2003. By that time, he was virtually unable to walk and asked for authorization for an electric scooter. Since this request was refused, he was referred to another neurologist who established the diagnosis based on magnetic resonance imaging (MRI) and significant improvement following a spinal tap test. A week later, a ventriculoperitoneal shunt was implanted, virtually restoring him to normal health.

Conn recognized the lack of awareness of NPH by many physicians and initiated a survey to explore the situation. Using a questionnaire, he interviewed 166 practicing physicians regarding their knowledge of NPH.^11,12 The interviewed candidates had graduated from 50 American and 33 international medical schools. Nearly one-third of the physicians had never heard of NPH, about 20% learned about NPH in medical school, and about 50% learned about it after medical school. One has to take into consideration that about one-half of the physicians graduated from medical school before NPH was described in the literature, which first occurred in 1965.

17.2 Educating the Public

Educating the public is as important as educating medical personnel. Similar to other endemic diseases in industrialized countries (e.g., hypertension, diabetes, stroke, cancer, Alzheimer disease), which are quite present in the public’s consciousness, there needs to be further education about NPH.

Low back pain catches public attention every other week in newspapers or the electronic media. Gait impairment with an onset beyond 65 years of age needs to have similar attention. “Is there a cure for dementia?” could be one of the provocative slogans to promote awareness of the Hakim triad.

“Public” not only means possible patients and their families but also nursing home staff or other inpatient or outpatient care facilities for the elderly population.

In his publication about his personal experience with the diagnosis and treatment of NPH, Conn discusses that the criteria used to define ventricular enlargement are difficult to establish.¹² Even more difficult to establish is the physician’s response to what is considered mild or moderate ventricular enlargement. The diagnosis may raise complex medical and socioeconomic issues about hospital stays, invasive diagnostic procedures, and even brain surgery. Educating the public as well as physicians should lead to an adequate and competent specialist evaluation of a patient with mild ventricular enlargement and moderate symptoms. Elderly patients with symptoms, as well as their families, should be given the opportunity to make an informed decision if they want a diagnostic evaluation and, if indicated, shunt surgery or not, whatever the risk. Conn points out that having experienced end-stage NPH and having been abandoned to his disease, he did not feel that there was much to lose by choosing surgery.¹²