1176 Case Reports / Journal of Clinical Neuroscience 20 (2013) 1176–1177 References 1. Priest JR, Magnuson J, Williams GM, et al. Cerebral metastasis and other central nervous system complications of pleuropulmonary blastoma. Pediatr Blood Cancer 2007;49:266–73. 2. Indolfi P, Casale F, Carli M, et al. Pleuropulmonary blastoma: management and prognosis of 11 cases. Cancer 2000;89:1396–401. 3. Hammad AM, Serag AR, Bayoumi SL. Pleuropulmonary blastoma: a case report. Internet J Thorac Cardiovasc Surg 2004; 6, http://dx.doi.org/10.5580/9ac. 4. Shivastava R, Saha A, Mehera B, et al. Pleuropulmonary blastoma: transition from type I (cystic) to type III (solid). Singapore Med J 2007;48:e190–2. 5. Corapcioglu F, Liman T, Aksu G, et al. A case report with type II pleuropulmonary blastoma: successful treatment with surgery and chemotherapy. Turk J Pediatr 2009;51:78–81. http://dx.doi.org/10.1016/j.jocn.2012.10.010 Apraxia of speech in healthy 36-year-old man Ciro Ramos-Estebanez a,⇑, Sankalp Gokhale b, Richard Goddeau c, Sandeep Kumar d a Department of Neurology, University of Miami, Miller School of Medicine, Jackson Memorial Hospital C215. 1120 NW 14th Street, Suite 1359-3, Miami, FL 33136, USA Neurocritical care, Division of Neurology, Department of Medicine, Duke University Hospital, Duke University School of Medicine, Durham, NC, USA c Department of Neurology, Vascular Neurology and Stroke Division, Dartmouth University School of Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, NH, USA d Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA b a r t i c l e i n f o Article history: Received 3 September 2012 Accepted 19 October 2012 Keywords: Apraxia Frontal Speech Stroke a b s t r a c t A 36-year-old healthy man presented with sudden onset speech difficulty. Thorough clinical examination revealed interesting deficits suggestive of apraxia of speech. He was found to have an infarct in his frontal region explaining the deficits. We have undertaken clinical evaluation and differential diagnoses of this condition. Ó 2012 Elsevier Ltd. All rights reserved. 1. Case report A 36-year-old right-handed man with a history of migraines developed an abrupt onset of inability to speak. His native language was Hebrew, but he was also fluent in English, and to some extent, Afrikaans. He was brought to the emergency room, where 30 minutes after symptom onset he was found to be in sinus rhythm with a blood pressure of 116/82 mmHg. His neurological examination showed marked impairment of speech output, although with effort he could articulate the initial phonemes of words; he accurately followed oral and written instructions. His writing and clock drawing were normal. He had minor difficulty with volitional tongue movements but no other sensory–motor deficits. A stroke protocol consisting of a head and neck CT scan/CT angiogram and CT perfusion scan performed 45 minutes after symptom onset was normal. A brain MRI performed 10 hours later revealed an acute infarct in the left frontal and subcortical regions (Fig. 1). Our patient experienced a significant improvement during his hospitalization (see Supplementary video taken on day 3). His mandibular, labial, lingual and palatal functions were normal. His speech evaluation was completed in three languages (Hebrew, Afrikaans and English) using the National Institutes of Health Stroke Scale language evaluation items. Word finding was intact, while naming was initially impaired. His speech was relatively fluent with a slow rate and significant pauses between words or syllables. There were brief instances of articulatory discoordination, and mild articulatory groping. He displayed reduced articulatory precision that triggered phonemic errors without a consistent pattern across all three languages. The phonemic precision and ⇑ Corresponding author. Tel.: +1 305 243 4621; fax: +1 305 243 7081. E-mail address: cramosestebanez@med.miami.edu (C. Ramos-Estebanez). prosody worsened with longer words. His insight about his deficits was preserved. Writing and spelling was appropriate. As expected from the limited nature of his insults, he recovered rapidly.1,2 2. Discussion The anatomical substrate of apraxia of speech (AoS) has been a matter of debate. Differences in the literature probably reflect individual variability in cortical gyral patterns,3 and cortical representations of the speech networks.2 More importantly, there has been a lack of consistency in the main nosological definitions of the diverse speech deficits in the literature.4 The current consensus localizes AoS to the dominant hemisphere. A network involving the left inferior motor cortex, the opercular aspect of the inferior frontal gyrus and its underlying white matter integrates simultaneous and sequential motor tasks required for proper articulation of words.5 The left anterior insular cortex (necessary in speech motor learning, not in speech production) was thought to be involved,6 yet this is probably not the case.2 Conversely, lesions at the medial subcallosal fasciculus and medial periventricular white matter (which convey information to premotor areas and the motor cortex representing the larynx, tongue, lips and facial muscles) may trigger AoS. Transcallosal disinhibition of the right motor and supplementary motor area seem to be the underlying mechanism in this patient.7 Finally, Broca’s area is involved in mild cases with transient deficits.2 As illustrated in our Supplementary video, patients with AoS hesitate and self-correct their constant phonemic errors. They may substitute phonemes and pronounce non-existent words fluently. Sometimes, there are deletions of a consonant at the beginning of a word starting with several consonants (for example, ‘‘tone’’ instead of ‘‘stone’’).8 The errors are inconsistent (a phoneme may be pronounced properly once then mistakenly twice, and Case Reports / Journal of Clinical Neuroscience 20 (2013) 1176–1177 1177 Fig. 1. AxialMRI showing acute infarct in left frontal region as (upper and lower left) a bright area on diffusion weighted imaging, and (upper and lower right) as corresponding correlation on an average diffusion coefficient map. those two times the sounds produced may be different). Besides, some phonemes do not sound like any phonemes in the patient’s language. In addition, transitions between syllables or phonemes may be difficult because specific aspects of the motor sequence required to produce the sounds is impaired. In this context, prosody will be abnormal. Along with these deficits, patients can experience brief periods of normal speech. AoS should be differentiated from: (i) dysarthria, (ii) aphasia, (iii) mutism or (iv) foreign accent syndrome. Patients with dysarthria constantly reproduce the same mistakes (which are variable in AoS). There is usually no hesitation, corrections, prosodic deficits or occasional intact sentences. In contrast to patients with sensory aphasia, those with AoS understand commands, produce meaningful speech, read (although their articulation is deficient) and write properly. When compared to Broca’s aphasia, AoS individuals are fluent. Moreover, deficits in AoS occur across languages, while patients with aphasia perform better in their primary language. In akinetic mutism, patients do not grope and struggle to produce words. Recovery from akinetic mutism after a left anterior cerebral artery stroke is typical for its hypophonic and hypokinetic articulation. Pseudo bulbar mutism presents with bulbar signs and symptoms such as pathological laughter/crying or facial weakness. Patients with foreign accent syndrome develop a pattern of speech characterized by an abnormal prosody and fixed phonemic and phonetic errors. These individuals do not exhibit the probing http://dx.doi.org/10.1016/j.jocn.2012.10.010 behavior seen in AoS when patients search for the proper way to pronounce a word. Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at http://dx.doi.org/10.1016/j.jocn.2012.10.010. References 1. Muellbacher W, Artner C, Mamoli B. The role of the intact hemisphere in the recovery of midline muscles after a recent monohemispheric stroke. J Neurol 1999;246:250–6. 2. Hillis AE, Work M, Barker PB, et al. Re-examining the brain regions crucial for orchestrating speech articulation. Brain 2004;127:1479–87. 3. Amunts K, Schleicher A, Burgel U, et al. Broca’s region revisited: cytoarchitecture and intersubject variability. J Comp Neurol 1999;412:319–41. 4. Mohr JP, Pessin MS, Finkelstein S, et al. Broca aphasia: pathological and clinical. Neurology 1978;28:311–24. 5. Schiff HB, Alexander MP, Naesser MA, et al. Aphemia: clinico-pathological correlations. Arch Neurol 1983;40:720–7. 6. Dronkers N, Ogar J. Brain areas involved in speech production. Brain 2004;127:1461–2. 7. Naeser MA, Palumbo CL, Helm-Stabrooks N, et al. Severe non-fluency in aphasia. Role of the medial subcallosal fasciculus and other white matter pathways in recovery of spontaneous speech. Brain 1989;112:1–38. 8. Aichert I, Ziegler W. Syllable frequency and syllable structure in apraxia of speech. Brain Lang 2004;88:148–59.