Shunt-responsive parkinsonism and reversible white matter lesions in patients with idiopathic NPH

BackgroundIdiopathic normal pressure hydrocephalus (iNPH) is a potentially treatable dementia and gait disorder with abnormal CSF dynamics.ObjectiveTo investigate and characterize the changes in motor symptoms and CT and MRI features of iNPH before and after a shunt operation using specific evaluation criteria.MethodsWe studied 17 definitive iNPH patients, diagnosed according to the clinical guidelines of both the Japanese Society of NPH and the International NPH Consultant Group, with ventricular enlargement (Evan’s index > 0.3) and narrowed CSF spaces at the high convexity on CT scan and /or MRI. The pre- and post-operative evaluation criteria for the gait and motor disturbances included the Japanese NPH Grading Scale-Revised (JNPHGSR), the Timed “Up and Go” test and the motor sections of the Unified Parkinson Disease Rating Scale. For cognitive impairments, the JNPHGSR, Mini Mental State Examination, Frontal Assessment Battery and Trail Making Test were used. White matter lesions were rated from the CT and/or MRI using a validated visual rating scale.ResultsAll patients showed specific CT and MRI findings, consisting of diffusely-dilated Sylvian fissure, as well as narrowed CSF space at the high convexity. Fifteen patients (88 %) showed white matter lesions on their CT or MRI images. These signs were ameliorated in all patients after the shunt operation. Evan’s index and the mean total scores on the visual scale for white matter lesions also improved significantly. Clinically, the patients had frequent parkinsonism (71 %), but relatively few had a history of either small-vessel diseases (29 %), hypertension (41 %) or diabetes (35 %). All patients showed gait disturbances, and these symptoms, including postural instability and body bradykinesia, improved significantly after the operation. Over half also showed signs of cognitive impairment and urinary incontinence, and all such symptoms and signs improved significantly.ConclusioniNPH often appears as a shunt-responsive type of parkinsonism and reversible white matter lesions among the geriatric population.

[1]  E. Yoshikawa,et al.  in vivo Presynaptic and Postsynaptic Striatal Dopamine Functions in Idiopathic Normal Pressure Hydrocephalus , 2007, Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism.

[2]  H. Budka,et al.  Topographical and cytopathological lesion analysis of the white matter in Binswanger’s disease brains , 2004, Acta Neuropathologica.

[3]  A. Richardson,et al.  Computerized axial tomography in idiopathic hydrocephalus. , 1979, Brain : a journal of neurology.

[4]  V. Preedy,et al.  Unified Parkinson's Disease Rating Scale , 2010 .

[5]  Marvin Bergsneider,et al.  Diagnosing Idiopathic Normal-pressure Hydrocephalus , 2005, Neurosurgery.

[6]  T. Mergner,et al.  Vascular risk factors and arteriosclerotic disease in idiopathic normal-pressure hydrocephalus of the elderly. , 1996, Stroke.

[7]  B. Messert,et al.  Syndrome of akinetic mutism associated with obstructive hydrocephalus , 1966, Neurology.

[8]  I. Akiguchi,et al.  Recovery Process of Gait Disturbance after Ventriculo-Peritoneal Shunt in Patients with Idiopathic Normal Pressure Hydrocephalus , 2007 .

[9]  J. Ojemann,et al.  Pathophysiology of parkinsonism due to hydrocephalus , 2004, Journal of Neurology, Neurosurgery & Psychiatry.

[10]  K. Mori,et al.  Management of idiopathic normal-pressure hydrocephalus: a multiinstitutional study conducted in Japan. , 2001, Journal of neurosurgery.

[11]  I Litvan,et al.  The FAB: A frontal assessment battery at bedside , 2000, Neurology.

[12]  S. Fahn.  Members of the UPDRS Development Committee. Unified Parkinson's Disease Rating Scale , 1987 .

[13]  Alteration of cerebral perfusion in patients with idiopathic normal pressure hydrocephalus measured by 3D perfusion weighted magnetic resonance imaging , 2005, Journal of Neurology.

[14]  A. Lang,et al.  Parkinsonian syndromes associated with hydrocephalus: Case reports, a review of the literature, and pathophysiological hypotheses , 1994, Movement disorders : official journal of the Movement Disorder Society.

[15]  N. Bargalló,et al.  Post-surgical changes in brain metabolism detected by magnetic resonance spectroscopy in normal pressure hydrocephalus: results of a pilot study , 2006, Journal of Neurology, Neurosurgery & Psychiatry.

[16]  K. Ishii,et al.  CSF spaces in idiopathic normal pressure hydrocephalus: morphology and volumetry. , 1998, AJNR. American journal of neuroradiology.

[17]  R. D. Adams,et al.  SYMPTOMATIC OCCULT HYDROCEPHALUS WITH "NORMAL" CEREBROSPINAL-FLUID PRESSURE.A TREATABLE SYNDROME. , 1965, The New England journal of medicine.

[18]  P. Scheltens,et al.  A New Rating Scale for Age-Related White Matter Changes Applicable to MRI and CT , 2001, Stroke.

[19]  H. Yamanouchi,et al.  Neurological signs and frontal white matter lesions in vascular parkinsonism. A clinicopathologic study. , 1997, Stroke.

[20]  M. Ishikawa.  Clinical guidelines for idiopathic normal pressure hydrocephalus. , 2004, Neurologia medico-chirurgica.

[21]  Diane Podsiadlo,et al.  The Timed “Up & Go”: A Test of Basic Functional Mobility for Frail Elderly Persons , 1991, Journal of the American Geriatrics Society.

[22]  R. D. Adams,et al.  The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics. , 1965, Journal of the neurological sciences.

[23]  Association between cognitive impairment and gait disturbance in patients with idiopathic normal pressure hydrocephalus , 2005 .

[24]  S. Stein,et al.  Normal-pressure hydrocephalus. , 1974, Lancet.

[25]  J. Krauss,et al.  Movement disorders in adult hydrocephalus , 1997, Movement disorders : official journal of the Movement Disorder Society.

[26]  W. Ehrenstein,et al.  Trail Making Test and visual search , 2004, Archiv für Psychiatrie und Nervenkrankheiten.

[27]  H. Chui,et al.  Subcortical ischaemic vascular dementia , 2002, The Lancet Neurology.

[28]  T Suenaga,et al.  Alterations in glia and axons in the brains of Binswanger's disease patients. , 1997, Stroke.

[29]  J. Krakauer,et al.  Reversible leukoencephalopathy associated with cerebral amyloid angiopathy , 2004, Neurology.

[30]  J. Krauss,et al.  White matter lesions in patients with idiopathic normal pressure hydrocephalus and in an age-matched control group: a comparative study. , 1997, Neurosurgery.

[31]  L. Mollinger,et al.  Age- and gender-related test performance in community-dwelling elderly people: Six-Minute Walk Test, Berg Balance Scale, Timed Up & Go Test, and gait speeds. , 2002, Physical therapy.

[32]  P. Bugalho,et al.  Normal-pressure hydrocephalus: White matter lesions correlate negatively with gait improvement after lumbar puncture , 2007, Clinical Neurology and Neurosurgery.

[33]  G. Battaglia,et al.  Different clinical and evolutional patterns in late idiopathic and vascular parkinsonism , 2005, Journal of Neurology.

[34]  A. Kluger,et al.  Alzheimer's disease comorbidity in normal pressure hydrocephalus: prevalence and shunt response , 2000, Journal of neurology, neurosurgery, and psychiatry.

[35]  S Ekholm,et al.  Normal pressure hydrocephalus: vascular white matter changes on MR images must not exclude patients from shunt surgery. , 2001, AJNR. American journal of neuroradiology.

[36]  M. Morris,et al.  Reliability of measurements obtained with the Timed "Up & Go" test in people with Parkinson disease. , 2001, Physical therapy.

[37]  G. Waldemar,et al.  Shunting Effects in Patients with Idiopathic Normal Pressure Hydrocephalus; Correlation with Cerebral and Leptomeningeal Biopsy Findings , 1999, Acta Neurochirurgica.

[38]  P. Black,et al.  Guidelines for management of idiopathic normal pressure hydrocephalus: progress to date. , 2005, Acta neurochirurgica. Supplement.

[39]  E. Rubio,et al.  Reappraisal of the intracranial pressure and cerebrospinal fluid dynamics in patients with the so-called “Normal pressure hydrocephalus” syndrome , 2005, Acta Neurochirurgica.

[40]  M. Poca,et al.  Good outcome in patients with normal-pressure hydrocephalus and factors indicating poor prognosis. , 2005, Journal of neurosurgery.

[41]  R. Katzman.,et al.  Syndrome of normal pressure hydrocephalus: possible relation to hypertensive and arteriosclerotic vasculopathy. , 1977, Journal of neurology, neurosurgery, and psychiatry.