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Letters: W E Owens and M S Okun Dystonia, tremor, and parkinsonism in a 54 year old man with 2-hydroxyglutaric aciduria J Neurol Neurosurg Psychiatry 2004; 75: 1362-1363 [Full text] [PDF]

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Palilalia in Parkinsonism: localizable to the globus pallidus?

Alberto J Espay, Alok Sahay and Fredy J. Revilla   (16 February 2006)

Palilalia in Parkinsonism: localizable to the globus pallidus? 16 February 2006
Alberto J Espay, Neurologist University of Cincinnati, Alok Sahay and Fredy J. Revilla Send letter to journal: Re: Palilalia in Parkinsonism: localizable to the globus pallidus? alberto.espay{at}uc.edu Alberto J Espay, et al.	Dear Editor, In the midst of our search for palilalia (pathological perseveration of words and phrases) in the context of Parkinsonism we encountered the interesting case report by Owens and Okun regarding a mentally retarded man with parkinsonism presumably due to 2-hydroxyglutaric aciduria, who had palilalia among other neurological deficits.[1] The main message, namely the consideration of urine organic acids testing in adults with atypical parkinsonian disorders, is somewhat undermined by the juvenile onset of the case (with “cerebral palsy” in the 20s). Nevertheless, we want to draw attention to an important clinical issue that this case raises. We should begin by addressing the relevant clinical question, namely, when do we test for organic acidurias in adults with Parkinsonism? It has been advised we should have a low testing threshold for these and other metabolic disorders when any signal abnormality is present in the basal ganglia, especially in children;[2] in the case of most organic acidurias, it implies hypointensity on T1W or hyperintensity on T2W and FLAIR sequences within the pallidum or putamen. We would not have tested for organic acidurias in an adult exclusively on the basis of white matter hyperintensities and ex-vacuo ventricular dilatation, as reported here. However, when reviewing the FLAIR images published, hyperintensities of the external segment of the globus pallidus (GPe) are indeed found (figure 1, left) and, remarkably, no comment is made of this finding, significant – one would have presumed – to the decision of proceeding to test for hydroxyglutaric aciduria. Pertinent to the scope of our initial search and unbeknown to Owens and Okun, their report may now be used to support a localizing value for palilalia. Kuoppamäki et el.[3] have recently studied an adult with Parkinsonism and palilalia resulting from alcohol-induced respiratory acidosis who, to our surprise, had isolated bilateral lesions of the GPe. As we have just learned, the very short list of palilalia associated with non-Parkinson’s disease parkinsonisms only includes the atypical variant (late onset with slow progression) of pantothenate kinase-associated neurodegeneration[4] and postencephalitic parkinsonism.[5] The GP suffers iron deposition in the former (with the classical eye-of-the-tiger appearance) and presumably viral-induced gliosis in the latter (in which the substantia nigra is the primary target).[6] Careful pathologic studies of the globus pallidus in postencephalitic parkinsonism indeed reflect predominant cell loss in the “outer segment... with relative normality of the inner segment of the pallidum” in all cases brought for postmortem assessment.[6] When assessing our next patient with palilalia, we will attend to its potential localizing value. References 1. Owens WE, Okun MS. Dystonia, tremor, and parkinsonism in a 54 year old man with 2-hydroxyglutaric aciduria. J.Neurol.Neurosurg.Psychiatry 2004;75:1362-3. 2. Gascon GG, Ozand PT, Brismar J. Movement disorders in childhood organic acidurias. Clinical, neuroimaging, and biochemical correlations. Brain Dev. 1994;16 Suppl:94-103. 3. Kuoppamaki M, Rothwell JC, Brown RG, Quinn N, Bhatia KP, Jahanshahi M. Parkinsonism following bilateral lesions of the globus pallidus: performance on a variety of motor tasks shows similarities with Parkinson's disease. J.Neurol.Neurosurg.Psychiatry 2005;76:482-90. 4. Hayflick SJ, Westaway SK, Levinson B, Zhou B, Johnson MA, Ching KH, Gitschier J. Genetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome. N.Engl.J.Med. 2003;348:33-40. 5. Sterling W. Palilalie et le symptome linguosalivaire dans le Parkinsonisme encephalitique. Rev.Neurol.(Paris) 1924;1:205-20. 6. Martin JP. The globus pallidus in post-encephalitic parkinsonism. J.Neurol.Sci. 1965;2:344-65.