Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 1 Atypical and potentially reversible types of dementia and cognitive dysfunctions: Normal Pressure Hydrocephalus Dr. med. Franziskos Xepapadakos Neurologist & Psychiatrist Deputy Head of Clienia Schlössli S.A., Private Psychiatric Hospital Oetwil am See / Zurich, Switzerland 9th Panhellenic Interdisciplinary Conference on Alzheimer's Disease and Related Disorders & 1 st Mediterranean Conference on Neurodegenerative Diseases Thessaloniki, 14-17 May 2015
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Atypical and potentially reversible types of dementia and ... · causing a subdural hematoma.-Undershunting may not have optimal improvement of the symptoms.-Shunt infections-Symptomatic
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Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 1
Atypical and potentially reversible types of dementia andcognitive dysfunctions:Normal Pressure Hydrocephalus
Dr. med. Franziskos XepapadakosNeurologist & PsychiatristDeputy Head of Clienia Schlössli S.A., Private Psychiatric HospitalOetwil am See / Zurich, Switzerland
9th Panhellenic Interdisciplinary Conference on Alzheimer's Disease and Related Disorders &1st Mediterranean Conference on Neurodegenerative DiseasesThessaloniki, 14-17 May 2015
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 2
Content
1.Definition
2. Etiology
3. Symptomatology
4.Diagnosis / Differential Diagnosis
5. Treatment
6. Case Report
7.Discussion
Normal Pressure Hydrocephalus
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 3
ΙΠΠΟΚΡΑΤΗΣ(Hippocrates)
detective
- crime!- motive?
- symptom!- disease?
physician
Normal Pressure Hydrocephalus
Normal Pressure Hydrocephalus (NPH) - general
Normaldruckhydrozephalus
past: uncritical indications, immature technology of valves, lowtherapeutic success, high complication rate.
present: modere diagnostic and therapeutic procedures, clinicalimprovement rates 70-90%, operative therapy superior in comparison toconservative or natural course.
Without therapy NPH leads within a few months to deterioration ofclinical findings and without surgery decreases the quality of life.
Today only 10-20% of the patients with an iNPH receive a specializedand professional therapy.
Normal pressure hydrocephalus (NPH) is often unrecognized.
Kiefer & Unterberg 2012
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki
Epidemiology
(Boschert et al., 2004)
Normal Pressure Hydrocephalus
•Although NPH is known for more than 40years epidemiological studies are rare.
•Prevalence is estimated at 0.1% amongindividuals aged ≥ 60 years.
•6% of nursing home residents may haveNPH. (Bejjani et al., 2005)
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 10
Etiology
•NPH can be:
• Idiopathic (ca. 2/3 of patients) or
• Secondary (ca. 1/3 of patients) to a multitude of insults (e.g.subarachnoid hemorrhage, meningitis, encephalitis, head trauma).
• NPH may develop years after the original injury!
•Despite intensive research, idiopathic NPH is still a mysterious entity ofunclear etiology.
Normal Pressure Hydrocephalus
(Bradley et al., 2004; Krauss & Halve, 2004)
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 11
Idiopathic NPH - Etiology
Theories on etiology of NPH:
Defective absorption of CSF (Bateman, 2000)
Deep white matter ischemia (Bradley et al., 1991)
Redistribution of vascular pulsations and decreased elasticity(compliance) of the brain parenchyma, vessels and subarachnoid spaces(Bateman, 2009)
Normal Pressure Hydrocephalus
(overview see Chrysikopoulos, 2009)
Pathophysiology
- The imbalance of CSF production and resorption is not caused by anincreased CSF production.
- Cause of the disease seems to be a limitation of the Windkessel effect(pressure reservoir function) of the basal cerebral arteries. As a result ofthat higher shear and compressive forces occur in the brainparenchyma. Because of physiological and physical differences mainly theperiventricular brain tissue gets damaged and reduced in volume.Through these focal brain damage results a ventricular dilatation.
- Another consequence is a decreased cerebral blood flow (highcoincidence of iNPH with disorders of cerebral circulation). Through thatlower CSF-turnover and lower clearance of toxic metabolics(coincidence of iNPH with Morbus Alzheimer).
Dr. F. Xepapadakos, Clienia Schlössli AG, Runder Tisch ZGPP, 09.04.2013 12
Kiefer & Unterberg 2012
Normal Pressure Hydrocephalus
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 13
Etiology
• Whatever the etiology, the result is a dilatation of the ventricles(ventriculomegaly). That implies a communicating hydrocephalus, where thearachnoid villi are unable to reabsorb CSF sufficiently without an obstruction toCSF flow in the ventricular system.
• The progressive ventriculomegaly may cause compression of structuresadjacent to the ventricles, resulting in the clinical manifestations of the disease.
• e.g. pressure on the frontal lobes: dementia symptoms
• e.g. pressure on cortical center for bladder control: incontinence
• e.g. pressure on corticospinal fibers lateral to ventricles: gait disturbance
Normal Pressure Hydrocephalus
(Hickey, 2003; Bejjani & Hammer, 2005)
Diagnosis - clinical features
Gait disturbance (up to 92%):- Slow, broad based and short-stepped gait- Shortened stride length, broad „magnetic“ gait (feet „sticking“ to
the floor)
- Balance disturbation- Difficulties in turning on the body‘s long axis- Relative less movement of the arms
Paulus W. et al. In: Diener H.-C. & Weimar C. 2012Lemke & Meier 2010
Normal Pressure Hydrocephalus
Diagnosis –clinical features and neuropsychological testing
Dementia (almost everyone):Type: subcortical dementia- lack of drive- psychomotor slowing- reduced attention and concentration performance- affective indifference- memory impairment (esp. of immediate and delayed recall [verbal
memory], but in comparison to Alzheimer’s disease with betterrecognition performance)
- apathy with loss of agility, spontaneity and communication- anxiety and depression
15
Paulus W. et al. In: Diener H.-C. & Weimar C. 2012
Normal Pressure Hydrocephalus
Duinkerke et al., 2004; Thomas et al., 2005; Iddon et al., 1999
Detrusor hyperactivity with partial or total absence of central inhibitorycontrol
The urinary incontinence is not expressed by the early dementia but by aneurogenic bladder dysfunction as an autonomic syndrome. Inaddition, the gait distrubance complicates a fast way to the toilet.
In later stages a frontal lobe syndrome disables the awareness of thedesire to urinate.
16
Paulus W. et al. In: Diener H.-C. & Weimar C. 2012
Normal Pressure Hydrocephalus
Kiefer & Unterberg 2012
Diagnostics – cerebral imaging (cCT / cMRI):Resorption disturbance of the CSF with:- Ventricular enlargement out of proportion to sulcal atrophy.- Rounding of the frontal horns.- Disproportion between the enlarged inner and rather narrow outer CSF spaces- Narrowed CSF spaces over the convexity near the vertex- Periventricular hypodensities (diffusion of CSF) in cCT and hyperdensity in T2-cMRI- Only low to moderate general brain atrophy or/and moderate vascular pathology which
does not explain the clinical symptoms.
(Arif, 2006)
17CT scan in NPH (Byrd, 2006) control patient with ventriculomegaly in NPH
Normal Pressure Hydrocephalus
18
Kiefer & Unterberg, Differentialdiagnose und Therapie des Normaldruckhydrozepahlus,Deutsches Ärzteblatt, Jg. 109, Heft 1-2, 9. Januar 2012
Diagnostics – cerebral imaging (cCT / cMRI):
Normal Pressure Hydrocephalus
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki
Diagnosis – Drainage of the CSF
The CSF tap test- Removal of approximately 30 to 70 mL of CSF via lumbar puncture.- The test is simple to perfom, wide spread, but however, controlled
studies are rare.- The single removal of CSF simulates a shunt-effect in advance.- The test considered positive if the symptoms (esp. gait disturbance)
temporarily improve after the removal of CSF and is - in addition to theclinical symptoms and the cerebral imaging – the most essentialcriterion for the diagnosis of NPH.
Kahlon et al., 2002; Hakim et al. 1965; Wikkelso et al. 1986; Bret et al. 1990; Malm et al. 1995a;Hebb & Cusimano 2001; Walchenbach et al. 2001; Paulus et al. In: Diener & Weimar 2012Kiefer & Unterberg 2012
Diagnosis – Drainage of the CSF (tap test)
- Assessment of gait improvement by measuring the time and distance- The test should be considered positive when walking time or walking
distance improve at least 20-30% and/or the cognitive performanceimprove at least 10%.
- Sensitivity of the CSF tap test 26-61% (Marmarou et al. 2005).
- Repeat of the test useful in unclear results (Gupta & Lang 2011).
Other technique:- External continuous lumbar drainage.
Prognosis:Better than dementia in Alzheimer’s disease(Improvement after shunt-implantation: gaitdisturbance> urinary incontinence> dementia)
Alternatively:Conservative therapy by intermittent therapeuticCSF-drainage in medical inoperable patients.
(Paulus W. et al. In: Diener H.-C. & Weimar C. 2012)(Bergsneider et al., 2005)
Normal Pressure Hydrocephalus
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 22
Normal Pressure Hydrocephalus
Therapy
Complications (today <20%, earlier 35-40%):
- Overshunting may result in ventricular collapse,
causing a subdural hematoma.
-Undershunting may not have optimal improvement
of the symptoms.
-Shunt infections
-Symptomatic epilepsy
-Acute or chronic subdural hematoma
-Shunt dysfunction
(Bergsneider et al., 2005)
(Paulus W. et al. In: Diener H.-C. & Weimar C. 2012)
Therapy – predictorsPredictors for positive results after shunt implantation:- Gait disturbance clinically predominant- Gait disturbance before cognitive decline- Short history esp. of the cognitive impairment- Mild to moderate cognitive deficits- Low to moderate lesions of the brain tissue in the MRI- Significant improvement after lumbar CSF-drainage
Predictors for negative results after shunt implantation:- Severe dementia- Cortical dementia- Pronounced cortical atrophy- Pronounced vascular leukencephalopathy
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki
Normal Pressure Hydrocephalus
Differential diagnosis
General results unlikely for idiopathic normal pressure hydrocephalus:
- intracranial pressure > 25 cm H2O
- Age < 40 years
- Asymmetric or only temporal findings
- Cortical deficits e.g. aphasia, apraxia, paresis
- Progressive dementia without gait disturbance and without
ventriculomegaly
29
Kiefer & Unterberg 2012
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki
Normal Pressure Hydrocephalus
NPH & Alzheimer
Patients with moderate-to-severe tau and Ab pathology demonstratedmore severe baseline impairment on a composite measure ofcognition and poorer performance postoperatively on NPH symptomseverity scales and measures of cognition. Finally, in sharp contrast toindividuals with less severe pathology, patients with moderate-to-severe pathology failed to demonstrated benefit on any studymeasures accessing gait, cognition, and incontinence at 4-month followup.
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 30
Normal Pressure Hydrocephalus
Hamilton et al., 2010:
Summary
- Normal Pressure Hydrocephalus (NPH) is a treatable type of dementiawith clinical improvement rates 70-90% by using modern diagnostic andtherapeutic methods.
- NPH is characterized by combination of clinical and radiologicalfeatures.
- Primary (idiopathic) NPH is distinguished from secondary NPH.- Typical clinical features (Hakim triad) are gait impairment, dementia
and urinary incontinence.- Gait impairment: short-stepped, broad based, slow, glue-footed walk with
externally rotated posture of feet and particular difficulty turning on the body‘slong axis.
- Dementia: psychomotor slowing, impaired attention and concentration, short-term memory impairement esp. of delayed recall, apathy, reduced drive.
- Urinary incontinence: detrusor hyperactivity with disturbed inhibitory control.
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 31
Normal Pressure Hydrocephalus
Kiefer & Unterberg 2012
Summary
- Cerebral imaging: disproportionate widening of the ventricles incomparison to the cerebral sulci (inner vs. outer CSF spaces) andsurrounding the outer surface of the brain (“tight convexity”) narrowsubarachnoid space
- Spinal tap test: lumbar puncure with removal of 30-70 mL of CSF.The testshould be considered positive when walking time or walking distanceimprove at least 20-30% and/or the cognitive performance improve at least10%.
- Standard treatment of NPH is the implantation of a venticuloperitoneal(VP) shunt. Clinical improvement rates of the surgery in studies aprox. 70-90%.
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 32
Normal Pressure Hydrocephalus
Kiefer & Unterberg 2012
Therapy of dementia – Improvement of Quality of Life
• impressive improvement of subjective wellbeing, patient felt moreawake 2 days long after CSF removal
→ admission to neurosurgery: VP shunt implantation
Case report 1
Medication:
Venlafaxine 75mg/d – drug level in blood 0.74 µmol/l(nausea/ vomiting with drug level of 112.5mg/d)
Lamotrigine 50mg/d
Case report 1 - outcome1 month after VP-shunt-implantation:- Progressive clinical improvement- Gait: fluent, large stepped. No tendency to fall anymore. Urinary
incontinence.- MMSE 30/30.
1 year after VP-shunt-implantation:- Further clinical improvement- Gait: secure, quickly, large stepped, no broad based, no tendecy to fall.- MMSE 30/30.
Patient lives alone, independently, without restriction in ADL’s, at herhome.
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 44
Case report 2Hospitalisation of the 79 year old woman:
Admission to our special unit for dementia from a somatic hospital
because of major deficits in performing activities of daily living,
disorientation and intermittent hallucinations.
Somatic, cardiologic as well as the red and white cell examination
was normal.
Normal Pressure Hydrocephalus
Case history:
In the months before admission she became increasingly
forgetful, often irritable and refusing. In addition she showed
cognitive and physical decline as well as social isolation.
Finally, she was obviously confused and physically deprived.
ReferencesAdams RD, Fisher CM, Hakim S, et al. Symptomatic occult hydrocephalus with ‘‘normal’’ cerebrospinal-fluid pressure. A
treatable syndrome. N Engl J Med 1965;273:117–26.Arif D. Normal pressure hydrocephalus. E medicine 2004. Available at: http://www.emedicine.com/NEURO/topic277.htmBateman GA. Vascular compliance in normal pressure hydrocephalus. AJNR 2000;21:1574–85.Bateman GA. The pathophysiology of idiopathic normal pressure hydrocephalus: Cerebral ischemia or altered venous
hemodynamics? AJNR 2008;29:198–203.Bech RA, Waldemar G, Gjerris F, et al. Shunting effects in patients with idiopathic normal pressure hydrocephalus:
correlation with cerebral and leptomeningeal biopsy findings. Acta Neurochir (Wien) 1999;141:633-639.Bech-Azeddine R, Waldemar G, Knudsen GM, et al. Idiopathic normal-pressure hydrocephalus: evaluation and findings in
a multidisciplinary memory clinic. Eur J Neurol 2001;8(6):601-11.Bejjani GK, Hammer MD. Normal-pressure hydrocephalus: another treatable dementia, part I. Contemporary
Neurosurgery. 2005;27(16):1-4.Bergsneider M, Black PM, Klinge P, Marmarou A, Relkin N. Surgical management of idiopathic normal-pressure
hydrocephalus. Neurosurgery 2005;57:S2-29–39.Bergschneider M, Egnor MR, Johnston M, et al. What we don't (but should) know about hydrocephalus. J Neurosurg (3
Suppl Pediatrics) 2006;104:157-9.Boschert S. Excess CSF can mimic Parkinson's disease, dementia: normal pressure hydrocephalus, typically
undiagnosed, can be treated with a shunt. Internal Medicine News. 2004;37(14):1-2.Bradley Jr WG, Whittemore AR, Watanabe A, Davis SJ, Teresi LM, Homyak M. Association of deep white matter infarction
with chronic communicating hydrocephalus: implications regarding the possible origin of normal pressurehydrocephalus. AJNR 1991;12:31–9.
Brean A, Eide PK. Prevalence of probable idiopathic normal pressure hydrocephalus in a Norwegian population. ActaNeurol Scand 2008;118:48–53.
Byrd C. Normal Pressure Hydrocephalus: Dementia's cause. The Nurse Practitioner 2006;31(7):28-35.Chrysikopoulos H. Idiopathic normal pressure hydrocephalus: Thoughts on etiology and pathophysiology. Medical
Hypotheses 2009;73:718-724.Clarfield AM. The reversible dementias: do they reserve? Ann Intern Med 1988;109(6):476-86.Clarfield AM. The decreasing prevalence of reversible dementias: an updated meta-analysis. Arch Intern Med
2003;163(18):2219-29.Duinkerke A, Williams MA, Rigamonti D, et al. Cognitive recovery in idiopathic normal pressure hydrocephalus after shunt.
Cogn Behav Neurol 2004;17(3):179-84.Egnor M, Zheng L, Rosiello A, Gutman F, Davis R. A model of pulsation in communicating hydrocephalus. Pediatr
Neurosurg 2002;36:281–303.Factora R, Luciano M. Normal pressure hydrocephalus: diagnosis and new approaches to treatment. Clin Geriatr Med
2006;22:645-657.Golomb J, Wisoff J, Miller DC, et al. Alzheimer’s disease comorbidity in normal pressure hydrocephalus: prevalence and
Dr. med. Franziskos Xepapadakos, PICAD & MeCoND 2015, Thessaloniki 53
ReferencesGreitz D. Radiological theories and implications for therapy. Neurosurg Rev 2004;27:145–65. assessment ofhydrocephalus: new
Hamilton R, Patel S, Lee EB, et al. Lack of shunt response oin suspected idiopathic normal pressure hydrocephalus withAlzheimer disease pathology. Ann Neurol 2010;68:535-540.
Hakim S, Adams RD. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure.Observations on cerebrospinal fluid hydrodynamics. J Neurol Sci 1965;2(4):307–27.
Hickey J. The Clinical Practice of Neurological and Neurosurgical Nursing. 5th ed. Philadelphia, Pa:Lippincott, Williams &Wilkins, 2003.
Iddon JL, Pickard JD, Cross JJ, et al. Specific patterns of cognitive impairment in patients with idiopathic normal pressurehydrocephalus and Alzheimer´s disease: a pilot study. J Neurol Neurosurg Psychiatry 1999;67(6):723-32.
Johanson CE. Choroid plexus – cerebrospinal fluid circulatory dynamics: impact on brain growth, metabolism and repair.In: Conn PM, editor. Neuroscience in medicine. Totowa, NJ: Humana Press; 2009. p. 173-200.
Kahlon B, Sundbarg G, Rehncrona S. Comparison between the lumbar infusion and CSF tap test to predict outcome aftershunt surgery in suspected normal pressure hydrocephalus. J Neurol Neurosurg Psychiatry. 2002;73:721-727.
Krauss JK, Halve B. Normal pressure hydrocephalus: survey on contemporary diagnostic algorithms and therapeuticdecision-making in clinical practise. Acta Neurochir (Wien) 2004;146(4):379-88 [discussion 388].
Levine DN. Intracranial pressure and ventricular expansion in hydrocephalus: have we been asking the wrong question? JNeurol Sci 2008;269:1-11.
Marmarou A, Young HF, Aygok GA, et al. Diagnosis and management of idiopathic normal-pressure hydrocephalus: aprospective study in 151 patients. J Neurosurg 2005;102:987–97.
Momjian S, Owler BK, Czosnyka Z, Czosnyka M, Pena A, Pickard JD. Pattern of white matter regional cerebral blood flowand autoregulation in normal pressure hydrocephalus. Brain 2004;127:965-72.
Silverberg G. Normal pressure hydrocephalus (NPH): ischemia, CSF stagnation or both. Brain 2004;127:947–8.Spetzler RF. Normal pressure hydrocephalus. Barrow Quarterly 2003;19:1Thomas G, McGirt MJ, Woodworth G, et al. Baseline neuropsychological profile and cognitive response to cerebrospinal
fluid shunting for idiopathic normal pressure hydrocephalus. Dement Geriatr Cogn Disord 2005;20(2-3):163-8.Vanneste JA. Diagnosis and management of normal-pressure hydrocephalus. J Neurol 2000;247(1):5-14.Verrees M., Selman W. Management of normal pressure hydrocephalus. Am Fam Physician. 2004;70:1071-1078.William G. Bradley, Francis G. Safar, Claudia Hurtado, Justin Ord & John F. Alksne. Increased Intracranial Volume: A Clue
to the Etiology of Idiopathic Normal-Pressure Hydrocephalus? American Journal of Neuroradiology 2004;25:1479-1484.
Kiefer & Unterberg 2012
ΕΥΧΑΡΙΣΤΩ ΠΟΛΥ ΓΙΑ ΤΗΝ ΥΠΟΜΟΝΗ ΚΑΙ ΤΗΝ ΠΡΟΣΟΧΗ ΣΑΣ!THANK YOU VERY MUCH FOR YOUR PATIENCE AND ATTENTION