NORMAL

PRESSURE
ANEL

HYDROCEPHALUS
MD

YSSA D'ABREAU,

Hakim and Adams first described the syndrome of Idiopathic Normal Pressure Hydrocephalus (IN PH) in
1965. 1,2In tWo articles they reported six

patients with a triad of "mild impairment of the memory, slowness and paucity of thought and action, unsteadiness of gait and unwitting urinary incontinence." In three out of the six patients rhe etiology was unknown, while head trauma (2) and a cystof the third ventricle accounted for the other three patients. Their observation was based on a syndrome, known at the time, of secondary hydrocephalus. In this disorder, hydrocephalus developed as a result of blood or inflammatory cells in the cerebrospinal fluid (CSF), blocking reabsorption. WIth expanding hydrocephalus due to increased pressure, patients would usually develop impaired consciousness, headache, and subsequent gait abnormality and bladder incontinence. Therefore, what was new was the normal CSF pressure and the idiopathic nature of the disease. In 1982, CM Fisher underscored the importance of the gait disturbance in the description of NPH. 3 He described 30 patients over 60 years of age, evaluated personally, who presented primarilywith gait disturbance, hydrocephalus on CT scan and responsiveness to CSF drainage, either by shunting or by spinal punctUre.None of the patients had dementia preceding the gait abnormality, although both problems could be present. He reported that the onset of gait abnormality before cognitive decline predicted a better prognosis after shuntmg. In 1986, Graff-Radford and Godersky correlated clinical symptoms and shunt responsiveness. 4 In their series of 21 patients shunted for INPH, 16 patients improved. Of these, 11 had a gait abnormality that preceded the dementia, while 5 had a simultaneous presentation. Of the 5 patients who did not improve, 3 had a dementing illness prior to a gait abnormality, 2 had a concomitant presentation and in only one did the

gait disturbance precede the dementia. Based on those ~ndings, the authors concluded that gait abnormality preceding the dementia was a good prognostic factor in the selection of patients for shuntmg. INPH is often considered one of the rare reversiblecauses of dementia, which iswhy it isso commonly discussed.However, its importance as a potential cause of reversible dementia is debatable. In a recent meta-analysis of 39 articles addressing reversibility in dementia, 7042 patients with cognitive impairment (5620 with dementia) were assessed. 5 Potential reversibility was found in 9% of the patients, of whom only 0.6% of the patients actually reversed. In 2.2% of these patients a surgically amenable condition was found, including NPH (idiopathic or secondary), subdural hematomas and cerebral tumors. According to this analysis, the estimated incidence of shunt-responsive NPH is 2.2 per million per year. Similar figures can be derived from memory clinic reports. 6In a stUdyof71 consecutive patients sent for evaluation of possible INPH, 36 patients did not meet criteria based on history, physical and neurological examination and neuroimaging. The most common diagnosis in the excluded population was cerebrovascular disease. Thirty-five patients were admitted to the neurosurgery ward for hydrodynamic studies, of whom 13 were considered shunt candidates. Only 8 patients respond to shunting. Considering shunt responsiveness as the only criterion to confirm the diagnosis of INPH, the calculated incidence of INPH in this population was 1.5 per million per year. The pathophysiology of INPH is unclear, and many theories have been proposed. It is important to differentiate primary from secondary cases of NPH. The latter usually develops after meningitis, subarachnoid hemorrhage or head trauma. Secondary cases have a much better prognosis and stUdies evaluating NPH should differentiate betWeenthose

tWo populations.

Although isolated intracranial pressure (ICP) measurements in INPH are within the normal range, continuous ICP monitoring demonstrates an increase in the occurrence of B-waves in these patients. 8While B-wavesare a physiological phenomena of unclear etiology,their presence in 50% or more of the recording time in NPH patients predicts a good shunting response according to some studies. The associationbetWeenINPH and hypertension, subcortical and periventricular white matter abnormalities (WMD) suggests a possible cerebrovascular role in the development of INPH. 9 The presence of WMD does not preclude shunting, however it carries a poorer prognosis. 10 Abnormalities in the cerebral blood flow (CBF) in the periventricular areas and subcortical white areas,mostly in the frontal lobes have been demonstrated in different studies utilizing multiple tech-

niques.II Someexpertsbelievethe alterations in CBF, also known as "misery perfusion", are responsible for the symptoms. Some reports have suggested reversibility or a decrease in those abnormalities after successfulshunting or CSF drainage. Unfortunately, the different techniques used in the different stUdies, may explain the variability of the results. Pathological changes at the arachnoid level were described as a potential culprit in the abnormalities in CSF flow, secondary to deficits in absorption. Biopsy stUdiesdo not corroborate this view. Pathological abnormalities in the arachnoid, when observed, do not correlate with results of CSF hydrodynamic studies or shunt responsiveness. 12In addition, deficits in absorption at the arachnoid levelwould not create a pressure gradient betWeen convexities and ventricles, and would therefore not lead to hydrocephalus. 7 Any doctor with geriatricexperience knows that the typical "clinical triad" is a relative common finding in the elderly. Many other diseaseprocesses,either alone

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or in combinarion, may produce a similar clinical picture. Some characreristics, as described below, are considered helpful in the differential diagnosis, although there . . is no . pathognomonic clinical or ImagIng sIgn. The gait abnormality in INPH is of "frontal dysequilibrium" or frontal gait disorder type. The most common symptoms described by patients are imbalance, tiredness oflegs, leg weakness and, as the disease progresses, shorter steps, shuffling, scuffing and slow turning. 1,2,3 A triad of hypokinetic gait, associatedwith a wide base, decreased step height and disturbance of dynamic equilibrium is considered "characteristic" ofINPH. 13 The differentiation between INPH and Parkinson's diseasecan be challenging. According to one study, in IN PH the most helpful gait parameters are the wide-based gait, with outward feet rotation, decreased range of motion of the lower extremities, with little or no reduction of arm swing. In contrast, PD usually has associated extrapyramidal features and the patients respond to visual and acoustic cues. 13 Cortical features such as apraxia, agnosia and aphasia are absent in INPH. Problems with attention, concentration, forgetfulness, apathy and mental slowing are the rule. Neuropsychologicaltests, although helpful in characterizing the deficit, are not good predictors of shunting outcome, and in most studies the improvement in extended neuropsychological batteries is minimal. 14 Iddon et al demonstrated the unpredictability of the cognitiveresponse to shunt. 15In this study they evaluated 11 patients with INPH, 5 in the demented range (mini-mental state examination-MMSE<24), and 6 with MMSE>24. After shunting, the demented patients demonstrated significant improvement in their MMSE and the Kendrick Object Learning Test (KOLT) which assessesrecall of everyday objects after a brief presentation period of a black-and-white illustration of them scores.On the other hand, the nondemented patients demonstrated decreasedverbal fluency, impaired spatial recognition and attentional set shifting task. Even though the subjects demonstrated improvement of gait after shunt-

ing, the impairment on tests sensitive to frontal dysfunction remained. Urinaty urgency is common in the earlier stages;incontinence is a later sign. 7 Urodynamic studies demonstrate hyperreflexia and instability of the bladder detrusor muscle, but there isno evidence of defectivebladder control. This suggests damage in the periventricular pathways to the sacralbladder center and decreased inhibition of bladder contractions.

IDIOPATHIC NORMAL PRESSURE

HYDROCEPHALUS IS AN OVERESTIMA UNCOMMON DISORDER, COMMON TRIAD WITH CLINICAL IN THE A TED,

CSF drainage by lumbar puncture, often considered a "gold standard" in the diagnosis ofINPH, has a vety high incidence of false negatives; and a prospectivestudy failedto provide any significant additional information compared to conservative clinical and neuroimaging criteria were utilized to select patients for shunting. 17Continuous CSF drainage poses a similar problem. In addition, it requires hospital admission, technical expertise, and is not always benign. 18 Cysternography does not improve the diagnostic accuracy of clinical and CT criteria combined, although many doctors still use it. 19Continuous intracranial pressure monitoring is a useful procedure and resultshave been uniform. Strong evidence supports that the frequent occurrence of B-waves (>50% of the recording time) in patients with INPH predicts a good response to shunting. 7 Unfortunately continuous ICP monitoring is not a routine procedure in most neurosurgical centers. There are many other diagnostic modalities, such as the infusion test, the conductance test, cerebrovascular and metabolic imaging studies. None has consistently demonstrated a significant improvement in predicting shunt-responsiveness. 7 The differential diagnosis of NPH includes Alzheimer's disease and related dementias, various causes of Parkinsonism, most notably Parkinson'sdisease, dementia with Lewy bodies (DLB) and "multi-infarct encephalopathy". Before considering the diagnosis ofINPH, one should exclude other possible explanations rather than the other way around. As mentioned before, INPH, is a relativelyrare disease,with an incidence comparable to Creutzfeld-Jacob disease,while Alzheimer's disease, Parkinson's disease, DLB and multi-infarct state are common pathologies. The predictors of a positive shunt response are gait predominant disorder that precedes any cognitive changes, a short histoty or absence of cognitive impairment, a known cause for the development of the hydrocephalus, absence of white matter lesion in neuroimaging studies. When available, the presence of B-waves for at least 50% of the recording time, improvement of symptoms af-

ELDERLY POPULATION.

The diagnosis of IN PH relies on clinical criteria, neuro-imaging evidence of hydrocephalus and ideally evidence of abnormalities in CSF hydrodynamics. Vanneste et al evaluated the predictive value of clinical criteria and CT in the diagnosis of INPH. 16By using strictly clinical (predominant gait disturbance, absent to mild cognitive deficit) and CT criteria (rounded frontal horns, moderate ventriculomegaly, absence of white matter diseaseand cortical atrophy), the positive predictive value (PPV) was 65%, and the negative predictive value (NPV) was 76%. Ineffective shunting was observed in 11% of the patients, while missed improvement occurred in 13%. More liberal criteria would have resulted in a PPV of 58%, ineffective shunting in 37% and missed improvement in 5% of the patients. The authors concluded that clinicalcriteria associated with appropriate neuroimaging is the standard by which new diagnostic testing should be used to improve diagnostic accuracy and shunt responsiveness.

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ter CSF drainage and CSF outflow resistance over 18mmH G/ ml per minute are also valuable prognostic factors. In contrast, severe dementia, dementia as the first symptom and severe atrophy or white matter diseaseon the MRI are predictors of a negative response to shunting 7. One may argue that it is appropriate to shunt a demented, gait-impaired patient, to improve the gait, even though n.o significant improvement will be expected in cognitive fimction. In that case, the family should have realistic expectations, and be informed of the risks of the shunt. The mean chance of significant improvement after shunting is 30 to 40% in INPH and 50-70% in secondary cases.Complications range between 20-40% of the cases, and include strokes, subdural hematomas, shunt malfunctioning, seizures and problems related to the anesthesia. Serious complications (death, severe residual deficit) usually arise in patients with severe co-morbidities, and are observed in less than 8% of the patients with INPH. 7,20 Dementia is the least likely symptom to improve especially if severe. In conclusion, INPH is an overestimated, uncommon disorder, with a common clinical triad in the elderly population. Potential improvement (but probably not reversibiliry) of the gait more than the cognitive dysfunction can be expected only in highly selected patients.

REFERENCES

1.
2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14.

Hakim S, Adams RD.] Neurol Sci 1965;2:307-27. Adam RD, FisherCM, Hakim S, et al. NE]Ml965;273:11726. Fisher CM. NeuroI1982;32:358-63. Graff-Radford NR, Godersky Jc. Arch NeuroI1986;43:9402. Clarfield M. Arch Intern Med2003;163:2219-29. Bech-Azeddine R, Waldemar G, Knudsen GM, et al. Eur] Neurol2001 ;6:279-88. Vanneste JAL.] NeuroI2000;247:5-14. Symon L, Dorsch NW Lancet 1972;2:1291-2. Krauss JK, Regel JP, Werner V, et al. Stroke 1996:27:24-9. Krauss JK, RegelJP, Vach W, et al. Neurosurg 1997;40:491-6. Owler BK, Pickard JD. Acta Neurol Scand 2001 ;104:325-42. Bech RA, Juhler M, Waldemar G, et al. Neurosurg 1997;40:497-502. Stoltze H, Kuhtz-Buschbeck JP, Drticke H, et al. ] Neurol Neurosurg Psychiatry 2001;70:289-97. Savolainen S, Hurskainen H, Paljarvi L, et al. Acta Neurochir 2002; 144:515-23.

15. Iddon JL, Pickard JD, Cross JL, et al.] Neurol Neurosurg Psychiatry 1999;67:723-32. 16. VannesteJ, Augustijn P,Tan WF, Dirven c.] NeurolNeurosurg Psychiatry 1993;56:251-6. 17. Maim J, Kristensen B, Karlsson T, et al. Arch Neurol 1995;52:783-9. 18. Walchenbach R, Geiger E, Thomeer RTWM, Vanneste JAL. ] Neurl Neurosurg Psychiatry 2002;72:503-6. 19. Vanneste J, Augustijn P, Gareth AG, et al. Arch Neurol 1992;49:366-70. 20. Vanneste J, Augustin P, Dirven C, et al. NeuroI1992;42:549.

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