WJNS  Vol.2 No.4 , November 2012
Structural neuroimaging and neuropsychological correlates of a single case of focal central pontine myelinolysis: Intact memory function with decreased intellectual and motor functions
ABSTRACT
We report on the case of middle-aged right-handed woman with central pontine myelinolysis (CPM) revealed by high resolution structural T2-weighted FLAIR MRI imaging. There was a general flattening of Wechsler Adult Intelligence Scale—Fourth Edition subtest scores which were 1 standard deviation below expected values. In contrast Wechsler Memory Scale—Fourth Edition visual and auditory memory scores remained within the normal range. Verbal working memory appeared mildly impaired while nonverbal working memory was not. Scores on the Advanced Clinical Solution’s Social Perception battery were all in the normal range as were academic skills measured by the Wide Range Achievement Test—Fourth Edition. Performance was impaired on the Delis-Kaplan Executive Function System’s counterpart of the Trail-Making Test: Part B. Similarly, on the Draw-A-Person Test there was a discrepancy in that our patient’s standard score was 76 compared to her estimated premorbid FSIQ in the average range. She also displayed bilateral motor coordination slowing on the Finger Tapping task collectively suggesting damage to pontine motor tracts. The Minnesota Multiphasic Personality Inventory—Second Edition—Restructured Form profile was consistent with a diagnosis of severe anxiety and depression perhaps due to damage to serotoninergic neural tracts originating within the central pons. Finally, the patient displayed severe sleep disturbances and other signs of reticular activating formation injury. CPM may constitute a unique means of studying reversible subcortical lesions in the central pons in otherwise healthy subjects with benign illness. To our knowledge this is among the first patients with CPM without the usual risk factors for the disorder and who was otherwise healthy. Knowledge of the etiology and neuropsychology of such patients might aid in understanding the interaction of the fronto-ponto-cerebellar tracts in executive functions and motor programming.

Cite this paper
Mulvogue, M. , Robinson, T. , Ijaz, M. and McCrea, S. (2012) Structural neuroimaging and neuropsychological correlates of a single case of focal central pontine myelinolysis: Intact memory function with decreased intellectual and motor functions. World Journal of Neuroscience, 2, 192-199. doi: 10.4236/wjns.2012.24030.
References
[1]   Adams, R.D., Victor, M. and Mancell, E.L. (1959) Central pontine myelinolysis: A hitherto undescribed disease occurring in alcoholic and malnourished patients. Archives of Neurological Psychiatry, 81, 778-780.

[2]   Lampl, C. and Yazdi, K. (2002) Central pontine myelinolysis. European Neurology, 47, 3-10. doi:10.1159/000047939

[3]   Ropper, A.H. and Samuels, M.A. (2009) Principles of neurology. 9th Edition, McGraw-Hill, New York.

[4]   Sterns, R.H., Thomas, D.T. and Herndon, R.M. (1989) Brain dehydration and neurologic deterioration after rapid correction of hyponatremia. Kidney International, 35, 69-75. doi:10.1038/ki.1989.9

[5]   Kleinschmidt, B.K., Masters,-De, and Norenberg, M.D. (1981) Rapid correction of severe hyponatremia causes demyelinization: Relation to central pontine myelinolysis. Science, 211, 1068-1070. doi:10.1126/science.7466381

[6]   Sterns, R.H. (1990) The management of symptomatic hyponatremia. Seminars in Nephrology, 10, 503-504.

[7]   Laureno, R. and Illowsky Karp, B. (1997) Myelinolysis after correction of hyponatremia. Annals of Internal Medicine, 126, 57-62.

[8]   Vexler, Z.S., Ayus, J.C., Roberts, T.P., Fraser, C.L., Kuchasczyk, J. and Arief, A.I. (1994) Hypoxic and ischemic hypoxia exacerbate brain injury associated with metabolic encephalopathy in laboratory animals. Journal of Clinical Investigation, 93, 256-264. doi:10.1172/JCI116953

[9]   Norenberg, M.D. (1983) A hypothesis of osmotic endothelial injury: A pathogenetic mechanisms in central pontine myelinolysis. Archives of Neurology, 40, 66-69. doi:10.1001/archneur.1983.04050020028004

[10]   Bonham, C.A., Diminguez, E.A., Fukui, M.B., Paterson, D.L., Pankey, G.A., Wagener, M.M., Fung, J.J. and Singh, N. (1998) Central nervous system lesions in liver transplant recipients: Prospective assessment of indications for biopsy and implications for management. Transplantation, 66, 1596-1604. doi:10.1097/00007890-199812270-00005

[11]   Rodriguez, J., Benito-Leon, J., Molina, J.A., Buckels, J. and McMaster, P. (1991) Central pontine myelinolysis associated with cyclosporine in liver transplantation. Neurologia, 13, 437-440.

[12]   Bird, G.L., Meadows, J., Goka, J., Polson, R. and Williams, R. (1990) Cyclosporin-associated akinetic mutism and extrapyramidal syndrome after liver transplantation. Journal of Neurology, Neurosurgery, and Psychiatry, 53, 1068-1071. doi:10.1136/jnnp.53.12.1068

[13]   Boon, A.P., Carey, M.P., Adams, D.H., Buckels, J. and McMaster, P. (1991) Central pontine myelinolysis in liver transplantation. Journal of Clinical Pathology, 44, 909- 914. doi:10.1136/jcp.44.11.909

[14]   Odier, C., Nguyen, D.K. and Panisset, M. (2010) Central pontine and extrapontine myelinolysis: From epileptic and other manifestations to cognitive prognosis. Journal of Neurology, 257, 1176-1180. doi:10.1007/s00415-010-5486-7

[15]   Mao, S., Liu, Z. and Ding, M. (2011) Central pontine myelinolysis in a patient with epilepsia partialis continua and hyperglycaemic hyperosmolar state. Annals of Clinical Biochemistry, 48, 79-82. doi:10.1258/acb.2010.010152

[16]   Vermetten, E., Rutten, S.J., Boon, P.J., Hofman, P.A. and Leentjens, A.F. (1999) Neuropsychiatric and neuropsychological manifestations of central pontine myelinolysis. General Hospital Psychiatry, 21, 296-302. doi:10.1016/S0163-8343(99)00018-3

[17]   Joseph, A.P. and Farmer, A. (1995) An unusual case of central pontine myelinolysis. Alcohol, 30, 423-425.

[18]   Goebel, H.H. and Herman-Ben Zur, P. (1972) Central pontine myelinolysis: A clinical and pathological study of 10 cases. Brain, 14, 495-504. doi:10.1093/brain/95.3.495

[19]   Ruchinskas, R. (2008) Cognitive dysfunction after central pontine myelinolysis. Neurocase: The Neural Basis of Cognition, 4, 173-179. doi:10.1212/WNL.43.11.2412-a

[20]   Arciero, S., Kempf, C., Bernard, F. and Gosselin, N. (2011) Cognitive and functional performance in daily activities before and after pontine and extrapontine myelinolysis: A case study. Neurocase, 1, 1-7.

[21]   Lee, T.M., Cheung, C.C., Lau, E.Y., Mak, A. and Li, L.S. (2003) Cognitive and emotional dysfunction after central pontine myelinolysis. Behavioural Neurology, 14, 103-107.

[22]   Price, B.H. and Mesulam, M.M. (1987) Behavioral manifestations of central pontine myelinolysis. Archives of Neurology, 44, 671-673. doi:10.1001/archneur.1987.00520180085025

[23]   McCrea, S.M. and Robinson, T.P. (2011) Visual puzzles, figure weights and cancellation: Some preliminary hypotheses on the functional and neural substrates of these three new WAIS-IV subtests. ISRN Neurology, 2011, 19. doi:10.5402/2011/123173

[24]   Lezak, M.D., Howieson, D.B. and Loring, D.W. (2004) Neuropsychological assessment. 4th Edition, Oxford University Press, New York.

[25]   Benson, N., Hulac, D.M. and Kranzler, J.H. (2010) Inde- pendent examination of the wechsler adult intelligence scale, 4th edition: What does the WAIS-IV measure. Psychological Assessment, 22, 121-130.

[26]   Folstein, M.F., Folstein, S.E. and McHugh, P.R. (1975) Mini-mental state: A practical method for grading the cognitive state if patients for the clinician. Journal of Psychiatric Research, 12, 189-198. doi:10.1016/0022-3956(75)90026-6

[27]   Strauss, E., Sherman, E.M. and Spreen, O. (2006) A compendium of neuropsychological tests: Administration, norms and commentary. Oxford University Press, New York.

[28]   Delis, D.C., Kaplan, E. and Kramer, J.H. (2001) Deliskaplan executive function system. The Psychological Corporation, San Antonio.

[29]   Reynolds, C.R. and Hickman, J.A. (2004) Draw-a-person intellectual ability test for children, adolescents, and adults. Pro-Ed Inc., Austin.

[30]   Beck, A.T., Steer, R.A. and Brown, G.K. (1996) Beck depression inventory. 2nd Edition, The Psychological Corporation, San Antonio.

[31]   Beck, A.T. and Steer, R.A. (1988) The beck hopelessness scale. The Psychological Corporation, San Antonio.

[32]   Beck, A.T. and Steer, R.A. (1993) Beck anxiety inventory. The Psychological Corporation, San Antonio.

[33]   Beck, A.T., Baruch, E., Balter, J.M., Steer, R.A. and Warman, D.M. (2004) A new instrument for measuring insight: The Beck cognitive insight scale. Schizophrenia Research, 68, 319-329. doi:10.1016/S0920-9964(03)00189-0

[34]   NCS Pearson (2009) Advanced clinical solutions for WAIS-IV and WMS-IV: Administration and scoring manual. The Psychological Corporation, San Antonio.

[35]   Ben-Porath, Y.S. and Tellegen, A. (2008) Minnesota Multiphasic personality inventory-2: Restructured form: Manual for administration, scoring and interpretation. University of Minnesota Press, Minneapolis.

[36]   Heilman, K.M. and Valenstein, E. (2003) Clinical neuropsychology. 4th Edition, Oxford University Press, New York.

[37]   Botez-Marquard, T., Bard, C., Leveille, J.and Botez, M.I. (2001) A severe frontal-parietal syndrome following cerebellar damage. European Journal of Neurology, 8, 347- 353. doi:10.1046/j.1468-1331.2001.00204.x

[38]   Rousseaux, M. and Steinling, M. (1992) Crossed hemispheric diaschsis in unilateral cerebellar lesions. Stroke, 23, 511-514. doi:10.1161/01.STR.23.4.511

[39]   Daum, I., Ackerman, H., Schugens, M.M., Reimold, C., Dicgans, J. and Birbaumer, N. (1993) The cerebellum and cognitive functions in humans. Behavioral Neuroscience, 107, 411-419. doi:10.1037/0735-7044.107.3.411

[40]   Kretschmann, H.J. and Weinrich, W. (2004) Cranial neuroimaging and clinical neuroanatomy: Atlas of MR imaging and computed tomography. 3rd Edition, Thieme, New York.

[41]   Kandel, E.R., Schwartz, J.H. and Jessell, T.M. (2000) Principles of neural science. 4th Edition, McGraw-Hill, New York.

[42]   Kolb, B. and Whishaw, I.Q. (1996) Fundamentals of human neuropsychology. 4th Edition, W.H. Freeman and Company, New York.

[43]   Madsen, K., Erritzoe, D., Mortensen, E.L., Gade, A., Madsen, K., et al. (2011) Cognitive function is related to fronto-striatal serotonin transporter levels—A brain PET study in young healthy subjects. Psychopharmacology (Berlin), 213, 573-581. doi:10.1007/s00213-010-1926-4

[44]   Anderson, D.E., Bell, T.A. and Awh, E. (2012) Polymorphisms in the 5-HTTLPR gene mediate storage capacity of visual working memory. Journal of Cognitive Neuroscience, 24, 1069-1076. doi:10.1162/jocn_a_00207

[45]   Stollstorff, M., Bean, S.E., Anderson, L.M., Devaney, J.M. and Vaidya, C.J. (2012) Rationality and emotionality: Serotonin transporter genotype influences reasoning bias. Social and Cognitive Affective Neuroscience (in press).

 
 
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