Dement Neurocogn Disord.  2016 Jun;15(2):49-51. 10.12779/dnd.2016.15.2.49.

Hydrocephalus in Patient with Multiple System Atrophy: Innocent Bystander or Guilty Party?

• Hwang I
• Ha SW
• Yang Y
• Kim SM

• KMID: 2442855
• DOI: http://doi.org/10.12779/dnd.2016.15.2.49

Abstract

BACKGROUND
Normal pressure hydrocephalus (NPH) is a poorly understood condition, which typically presents with the triad of gait disturbance, urinary incontinence and cognitive decline. Diagnosis of NPH is often challenging due to its varied presentation and overlap with other neurodegenerative diseases including multiple system atrophy (MSA).
CASE REPORT
A 68-year-old male developed rapidly progressive gait difficulty, urinary incontinence and memory impairment. Neurologic examination showed parkinsonism affecting the right side and impaired postural reflexes. Brain MRI showed enlargement of the ventricles and narrowing of the high convexity cerebrospinal fluid (CSF) spaces with relative dilated Sylvian fissure, the supporting features of NPH. 18F-fluorinated-N-3-fluoropropyl-2-b-carboxymethoxy-3-b-(4-iodophenyl) nortropane (¹â¸F-FP-CIT) PET showed decreased FP-CIT binding in the left posterior putamen and ¹â¸F-fluorodeoxyglucose PET showed decreased metabolism in the left basal ganglia, consistent with findings of MSA. CSF removal was performed and the symptoms were improved. The patient underwent ventriculo-peritoneal shunt and his gait and cognition improved.
CONCLUSIONS
NPH is a potentially treatable neurological disorder. Therefore, it is necessary to consider the possibility of accompanying NPH when hydrocephalus is present in other neurodegenerative diseases.

Keyword

multiple system atrophy; normal pressure hydrocephalus; parkinsonism

MeSH Terms

Aged
Basal Ganglia
Brain
Cerebrospinal Fluid
Cognition
Diagnosis
Gait
Humans
Hydrocephalus*
Hydrocephalus, Normal Pressure
Magnetic Resonance Imaging
Male
Memory
Metabolism
Multiple System Atrophy*
Nervous System Diseases
Neurodegenerative Diseases
Neurologic Examination
Parkinsonian Disorders
Putamen
Reflex
Urinary Incontinence
Ventriculoperitoneal Shunt

Figure

• 

  Fig. 1 Fluid-attenuated inversion recovery image of the patient. Enlargement of the ventricles and disproportion between narrowing of the CSF spaces at the high convexity and dilatation of the Sylvian fissures (A and B) were consistent with the imaging features of NPH. There was no atrophy of the putamen, middle cerebellar peduncle, pons, or cerebellum, as seen in MSA (C and D). CSF: cerebrospinal fluid, MSA: multiple system atrophy, NPH: normal pressure hydrocephalus.

• 

  Fig. 2 18F-FP-CIT PET and 18F-FDG PET images of the patient. A: 18F-FP-CIT PET image showed asymmetrically decreased FP-CIT binding in the left posterior putamen. B: 18F-FDG PET image demonstrated decreased metabolism in the left basal ganglia and preserved metabolism in the brainstem and cerebellum. 18F-FDG PET: 18F-fluorodeoxyglucose positron emission tomography, 18F-FP-CIT PET: 18F-fluorinated-N-3-fluoropropyl-2-b-carboxymethoxy-3-b-(4-iodophenyl) nortropane positron emission tomography.

Reference

1. Hakim CA, Hakim R, Hakim S. Normal-pressure hydrocephalus. Neurosurg Clin N Am. 2001; 12:761–773. ix

2. Gilman S, Wenning GK, Low PA, Brooks DJ, Mathias CJ, Trojanowski JQ, et al. Second consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008; 71:670–676.

3. Leinonen V, Koivisto AM, Savolainen S, Rummukainen J, Sutela A, Vanninen R, et al. Post-mortem findings in 10 patients with presumed normal-pressure hydrocephalus and review of the literature. Neuropathol Appl Neurobiol. 2012; 38:72–86.

4. Cabral D, Beach TG, Vedders L, Sue LI, Jacobson S, Myers K, et al. Frequency of Alzheimer's disease pathology at autopsy in patients with clinical normal pressure hydrocephalus. Alzheimers Dement. 2011; 7:509–513.

5. Curran T, Lang AE. Parkinsonian syndromes associated with hydrocephalus: case reports, a review of the literature, and pathophysiological hypotheses. Mov Disord. 1994; 9:508–520.

6. Magdalinou NK, Ling H, Smith JD, Schott JM, Watkins LD, Lees AJ. Normal pressure hydrocephalus or progressive supranuclear palsy? A clinicopathological case series. J Neurol. 2013; 260:1009–1013.

7. Lee WJ, Wang SJ, Hsu LC, Lirng JF, Wu CH, Fuh JL. Brain MRI as a predictor of CSF tap test response in patients with idiopathic normal pressure hydrocephalus. J Neurol. 2010; 257:1675–1681.

8. Stankovic I, Krismer F, Jesic A, Antonini A, Benke T, Brown RG, et al. Cognitive impairment in multiple system atrophy: a position statement by the Neuropsychology Task Force of the MDS Multiple System Atrophy (MODIMSA) study group. Mov Disord. 2014; 29:857–867.