Aphemia resulting from a left frontal hematoma Article abstract-A 15-year-old right-handed woman had selective impairment of speech-aphemia-after drainage of a left frontal hematoma caused by an arteriovenous malformation. There was no buccofacial or pharyngeal muscle dysfunction. Computerized tomography demonstrated residual injury extending from the Broca area to the inferior left precentral gyrus. Aphemia may have resulted from disruption of the connection between Broca's area and the portion of the motor cortex that controls oral and pharyngeal muscles. NEUROLOGY (Ny) 31: 353-356, March 1981 Robert L. Ruff and Ehud Arbit paresis, with the arm most severely involved. Only Aphemia is a disorder of language expression slight facial paresis was evident on voluntary or emoin which writing is normal but speech is impaired tional facial expression. She had full voluntary control despite adequate oropharyngeal muscular funcof respiration. Lingual and pharyngeal muscles functi0n.l-2~ This disorder is also referred to as simple aphasia,l cortical ~ ~ ~ ~ ~ ~ ~ ~ ~ , 2 ~ 4 ~ 5 . 1 0 ~ 1 1 ~ tioned 1 3 - 1 5normally, ~ 1 7 ~ 1 8 and ~ 2 3 she did not display ideomotor apraxia.22She performed 36 dictated commands sepaataxic aphasia:" subcortical motor aphasia,6J3 rately and in groups of four, involving the head, eyes, and pure word r n ~ t i s m . 1 0 ,The ~ ~ disorder ,~~ usually eyelids, lips, tongue, and all extremities. She could results from a cerebral infarct. The patients are swallow, cough, and cluck her tongue. initially mute, with subsequent incomplete recovThe patient was mute but not aphonic. She could hum ery of speech characterized by impaired cadence the tunes of popular songs correctly. She did not utter a sound when she tried to speak, but i t was possible to and articulation with normal syntax, vocabulary, understand her with lip reading. Penmanship with the and grammar. We describe a patient who became right hand was poor but legible. She wrote in full, gramaphemic as a result of left frontal lobe hemorrhage matically correct sentences, but she occasionally miswith residual damage to Broca's area, the left fronspelled words (WOof 300 words) and occasionally mistal operculum, and inferior precentral gyms. placed the syllables of a multisyllable word. Auditory Case report. This right-handed woman presented at age 15 years with a 2-hour history of progressive rightsided weakness, left frontal headache, and mutism. On examination she was lethargic, and a spastic right hemiparesis was most prominent in the arm. Although mute, she had voluntary control of respiration and could articulate well enough for one to read her lips. She could also respond correctly to questions by printing single words with her left hand, but she made spelling errors and would leave out prepositions or connectives when she tried to write sentences. She could identify objects correctly by pointing to the correct name from a list or by writing the name. Computerized tomography (CT) revealed a hematoma extending from the left posterior premotor frontal cortex to the inferior precentral gyrus (figure 1). The hematoma was surgically drained. At surgery, the hematoma was centered in the left middle frontal gyrus and dissected into the white matter of the left inferior frontal and inferior precentral gyri. Postoperatively, an arteriogram revealed an arteriovenous malformation (AVM) in the midportion of the operculofrontal branch of the left middle cerebral artery (figure 2). The AVM was resected 2 weeks after the hematoma was drained. At surgery, the AVM was restricted to the left middle frontal gyrus. The postoperative arteriogram revealed no mass effect or vessel spasm (figure 3). After the hematoma was drained, the patient's language function was studied serially, using the Minnesota test for differential diagnosis of aphasia.24 After the first surgery and during the first week after resection of the AVM, she had a moderate spastic right hemi- Figure 1 . CT scan without intravenous contrast injection demonstrating a left frontal hematoma. March 1981 NEUROLOGY (Ny) 31 353 Figure 2. Cerebral arteriogram, lateral projection, demonstrating a n AVM in the midportion of the operculofrontal branch of the left middle cerebral artery. Figure 4. CT scan without intravenous contrast injection performed 3 months after the AVM resection. There is residual injury i n the left frontal lobe extending from Broca’s area to the inferior precentral gyrus. and reading comprehension were normal. Spontaneous writing ability, facility in writing to identify objects, and ability to copy printed and dictated text were normal. The patient had a normal psychiatric evaluation, 354 NEUROLOGY (Ny) 31 March 1981 Figure 3 . Cerebral arteriogram, lateral projection, performed after resection of the left frontal AVM. and she could not speak during a n Amytal interview,2 5 Three weeks after the AVM resection, the patient and her parents thought that her motor function had returned to normal, but there was still slight right arm weakness, increased tone, and hyperreflexia. Penmanship returned to the premorbid state, and she rarely made spelling errors (3%of 150 words). At this point, she began to speak. Her first words were barely comprehensible grunts associated with much facial contortion. During the next 2 weeks articulation improved, with slow staccato cadence, transient word blocking, A list of monosyland phonetic disintegration.18,23.26-28 labic words with equal occurrence of phonemes in the initial, middle, and final position was used to test phonetic disability.28Articulation errors usually occurred at the initial position of words and most commonly with consonant phonemes, especially fricative, affricative, and cluster phonemes. Front consonants were not better pronounced than middle or back consonants. Phonemic substitution occurred rarely, and ?C%O of the substitutions were between similar phonemes. She named objects correctly. Repetitive speech was better than spontaneous speech, and she sang familiar songs with little difficulty. Two months after the AVM removal, the only residual motor deficit was slight right-arm hyperreflexia and increased tone. Speaking cadence remained slow, with occasional transient word blocking, but she no longer had difficulty with articulation. She had no problem expressing complex concepts verbally despite her languid speech. She stated that her thoughts “came more slowly in speaking than in writing,” and that verbal tasks requiring no thought, such as repetition or singing familiar songs, could be done with little hesitation. In school, the patient won her grade-level spelling bee, and her reading level was 2 years above grade level. The verbal deficit was unchanged when she was tested repeatedly over the subsequent 2 years. CT 3 months after the AVM resection showed residual damage extending from Broca’s area to the left frontal operculum and inferior precentral gyrus (figure 4). Discussion. CritchleyZ1cautioned that before accepting a diagnosis of aphemia it is necessary to establish that the patient is not suffering from hysteria29 or buccolingual a p r a ~ i a On . ~ ~psychiatric evaluation and psychologic testing, our patient showed no evidence of conversion reaction; the inability to speak during an Amytal interview indicated that the speech disorder was not “funct i ~ n a l . She ” ~ ~did not have buccolingual weakness or apraxia on formal testing.24Her lip movements permitted lip-reading, which indicated intact labial articulation. During recovery, the patient transiently displayed phonetic disintegration, an articulatory disorder found i n patients with expressive aphasia.1823 Phonetic disintegration differs from simple dysarthria in the specificity of the speech errors.28The discrepancy between the patient’s difficulty with spontaneous speech and her facility in repeating or singing suggested that she did not have a pure articulatory disorder. The pattern and time course of recovery of language function in our patient was similar to that described by Mohr31 in three patients with motor aphasia. Our patient differs from those described by Mohr31 in that she showed a greater degree of language recovery and did not have residual oropharyngeal weakness or apraxia. This difference may result from the more extensive lesions found in Mohr’s31patients. Their lesions extended deeper into the central white matter. K l e i ~ suggested t~~ that the severity of expressive aphasia was related to the extent of disruption of intrahemispheric and transcallosal connections to Broca’s area. These connections are also important in apraxia.22 The writing difficulty that our patient had before drainage of the hematoma may have resulted from compression of the white matter deep to Broca’s area. Broca initially used “aphemia” to refer to expressive language disorders involving both speech and writing.33 Trousseau34 then suggested that “aphasia” was a more appropriate term for language disorders, and reserved aphemia for isolated speech deficits.’ In some cases of expressive aphasia, speech was more impaired than writing.11,1735Marie11reported 17 cases of cerebral missile injuries that caused major speech difficulty and only minor writing impairment. The responsible lesions involved the left posterior second and third frontal gyri and the left inferior precentral gyrus.ll Mohr et a136 suggested that infarction of Broca’s area alone causes transient mutism. “Aphemia” has also been attributed to lesions of the left supplementary motor area.37*2Such patients are disinclined to speak rather than unable to speak, and are not truly aphemic because writing is also impaired.3742 T h e r e a r e 1 6 r e p o r t e d cases of t r u e aphemia,35-10.14,18202399 including a single case of pure phonetic d i ~ i n t e g r a t i o n . ~The ~ ” ~speech disorders varied from m ~ t i s m ~ ~to” Omoderate articulatory impairment.1823 Of the seven autopsies, one demonstrated a pontine infarct in addition to cerebral lesions.8 The lesions were restricted to Broca’s area in three or involved the inferior left precentral gyrus in three ~ t h e r s Therefore, . ~ ~ ~ ~the~ CT ~ evidence in our case of residual damage extending from Broca’s area to the region of the inferior precentral gyrus was compatible with reported autopsy findings. This is the first case of aphemia produced by a hematoma. Broca’s area was distinct from the AVM, therefore, it is unlikely that the AVM displaced the motor speech function to another area. Aphemia may result from disruption of the connections between Broca’s area and the portion of motor cortex controlling oropharyngeal muscle function,3~6-8~14,16.18-z3 whereas the complete syndrome of Broca aphasia requires a more extensive lesion.2232 36 From the Departments of Neurology (Dr. Ruff? and Neurosurgery (Dr. Arbit), the New York Hospital-Come11 Medical Center, New York, NY. Accepted for publication J u n e 10, 1980 Address correspondence and reprint requests to Dr. Ruff, Department of Medicine, Division of Neurology RG-20, University of Washington, Seattle, WA 98195. References 1. Baillarger JGF: De l’aphasie aupoint de vue psychologique: Aphasie simple: Aphasie avec perversion de la faculte du langage. In Baillarger JGF: Recherches sur les Maladies Mentales. Paris, Masson, 1865, vol 1, pp 584-601 2. Trousseau A: Clinique Medicale, 1865, vol 2, pp 571-626 3. Kussmaul A: Die Storungen der Sprache. Leipzig, Vogel, 1877, pp 581-875 4. Charcot JM: Differente formi dafazia: lezoni fatte nella salpetriere nel semestre destate del l’anno 1883. Milan, Antica Cam, 1883 5. Marie P: L’aphasie. La Revue de Medecine (Paris) 2693702, 1883 6. Lichtheim L: On aphasia. Brain 7:433-484, 1885 7. Bastian HC: On different kinds of aphasia with special reference to their classification and ultimate pathology. Br Med J 2:931-936, 985-990, 1887 8. Bastian HC: A Treatise on Aphasia and Other Speech Defects. London, Lewis, 1898 9. Von Monakow C: Gehirnpathologie. Vienna, Alfred Holder, 1904 10. Dejerine J L‘aphasie motrice: Sa localisation et son anatomie pathologique. Presse Medicale 14:453-457, 1906 11. Marie P Revision de la question de l’aphasie: La troisieme circonvolution frontale gauche ne joue aucun rble d a m la fonction du langage. 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Petit-Dutaillis D, Guiot G, Messing R, et a 1 Apropos d u n e aphemie par atteinte de la zone matrice supplementaire de Penfield. Rev Neurol (Paris) 90:95-106, 1954 39. Arseni C, Botez MI: Speech disturbances caused by tumors of the supplementary motor area. Acta Psychiatr Neurol &and 36:279-299, 1961 40. Botez MI: Clinical contribution to the study of the tumoral frontal syndrome. Psych Neurol (Basel) 140:347-368,1960 41. Botez MI, Carp N: Nouvelles donnees sur le probleme du mechanisme de declenchement de la parole. Rev b u m Neurol 5:153-158, 1968 42. Botez MI, Barbeau A: Role of subcortical structures, and particularly of the thalamus, in the mechanisms of speech and language. Int J Neurol 8:300-320, 1971 Article abstract-Acute chorea in a child followed ingestion of pemoline mesylate. In guinea pigs, in a n experimental model of chorea, chronic administration of pemoline induced behavioral supersensitivity to other dopaminergic agonists. Pemoline is similar to both d-amphetamine and methylphenidate in altering central dopaminergic sensitivity, and may cause chorea by similar mechanisms. Chronic pemoline therapy may offer no significant advantage over therapy with other indirect dopamine agonists. NEUROLOGY (Ny) 31: 356-360, March 1981 Paul A. Nausieda, M.D., William C. Koller, M.D., Ph.D., William J. Weiner, M.D., and Harold L. Klawans, M.D. Pemoline mesylate (Cylert) is a central stimulant used to treat children with minimal brain dysfunction.’ Dyskinetic movements may follow pemoline therapy, but a causal relationship has been debated.3 We describe a case of chorea associated with pemoline administration, and we also studied the effects of pemoline i n a n experimental model of chorea. Case report. This girl, age 2 years 3 months, was well until the day of admission, when she ingested an unknown quantity of pemoline tablets (37.5 mg) prescribed for a paternal cousin. About 30 minutes after ingestion, she was overactive, with intermittent tongue protrusion, irregular respiration, and rapid movements of the 356 NEUROLOGY (Ny) 31 March 1981 fingers. On examination she was “jittery” and spoke in a hoarse whisper. She was otherwise alert and appeared anxious and slightly flushed. General physical examination was unremarkable. Neurologically, she demonstrated generalized hyperreflexia, intermittent tongue protrusion, and increased salivation. She was treated with gastric lavage and 15 gm activated charcoal, which yielded one partially digested pemoline tablet. Thirty minutes later, her temperature was 38.5” C, pulse was 160, and blood pressure was 130/48 mm Hg. Her weight was 14.1 kg and her height 66 cm. She responded to her name but did not maintain gaze and failed to recognize her mother. She was restless, vigilant, and agitated, frequently responding by loud screams and purposeless intermittent flinging move- Aphemia resulting from a left frontal hematoma Robert L. Ruff and Ehud Arbit Neurology 1981;31;353 DOI 10.1212/WNL.31.3.353 This information is current as of March 1, 1981 Updated Information & Services including high resolution figures, can be found at: http://n.neurology.org/content/31/3/353.full.html Citations This article has been cited by 1 HighWire-hosted articles: http://n.neurology.org/content/31/3/353.full.html##otherarticles Permissions & Licensing Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://n.neurology.org/misc/about.xhtml#permissions Reprints Information about ordering reprints can be found online: http://n.neurology.org/misc/addir.xhtml#reprintsus Neurology ® is the official journal of the American Academy of Neurology. 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