Objective: Akinetic mutism (AKM) is an uncommon disorder with a complex neuropathology. There is no generally accepted treatment, and it is not known if late treatments are effective. The relationship between AKM and abulia is uncertain. Methods: The effects of dopaminergic treatment of a patient with chronic AKM after discrete bilateral infarctions of the mesencephalic ventral tegmental area and the lat eral hypothalamus were studied with motor measures, the Functional Independence Measure (FIM), and neuropsychological tests. Results: Treatment with a combination of carbidopa/levodopa and pergolide produced prompt amelioration of AKM with dra matic and rapid improvement in FIM. An apathetic, amotivational state persisted de spite resolution of akinesia and normal frontal executive functions. Conclusions: AKM may respond to dopaminergic treatment even after months of severe akinesia. The mechanism of abulia is more complex than simply a partial dopaminergic deficiency state and may persist even when AKM is treated and frontal cognitive functions are normal. Key Words: Akinetic mutism—Dopamine—Abulia.
References
1.
Alexander MPDisturbances in language initiation mutism and its lesser forms In Young R, Josephs AB, eds Movement disorders m neurology and neuropsychiatryOxford. Blackwell, 1992.389-96.
2.
Fisher CMHonored guest presentation abulia minor vs agitated behaviorClin Neurosurg1985;31.9-31.
3.
Stmeman MG, Escarce JJ, Gom JE, Hamilton BB, Granger CV, Williams SVA case mix classification system for medical rehabilitationMed Care1994;32:366-79.
4.
Ferbert A., Thron A.Bilateral anterior cerebral atery territory m-farction in the differential diagnosis of basilar artery occlusionJ Neurol1992;239:162-4.
5.
Laplane D., Talaraich J., Meininger V., Bancaud J., Orgogozo JMClinical consenquences of coticectomeis involving the supplementary motor area in man. J Neurol Sci1977;34301-14.
6.
Lechevalier B., Bertran F., Busson P., Chapon F., Raoul G., De La Sayette V.Akmetic mutism with right hemiplegia caused by infarction m the territory of the left anterior cerebral artery. Rev Neurol (Pans), 1996,152.181-9.
7.
Minagar A., David NJBilateral infarction m the terntory of the anterior cerebral arteries (see comments. Neurology1999,52: 886-8.
8.
Barns RW, Schuman HRBilateral anterior cingulate lesions syndrome of the anterior cingulate gynNeurology1953,344-52.
9.
Devinsky O., Morrell MJ, Vogt BAContributions of the anterior cingulate cortex to behaviorBrain1995,118.279-306.
10.
Nielsen JM, Jacobs LLBilateral lesions of the anterior cingulate gyri. Bull LA Neurol Soc1951;6:231-4
11.
Cairns H.,Oldfield RC, Pennybacker JB, Whitteridge D.Akinetic mutism with an epidermoid cyst of the third ventncleBrain1941;64:273-90.
12.
Ross ED, Stewart RMAkmetic mutism from hypothalamic damage. successful treatment with dopamine agonistsNeurology1981,31.1435-9
13.
Anderson B.Relief of akmetic mutism from obstructive hydrocephalus using bromocriptine and ephedrine: case reportJ Neurosurg1992;76.152-5
14.
Berger L., Gauthier S., Leblanc R.Akinetic mutism and parkinsonism associated with obstructive hydrocephalus . Can J Neurosci1985,12255-8
15.
Moser A., Freyberger HJ, Bruckmann H., Kompf D.Akinetischer mutismus bei dekompensiertem triventrikularen hudrozephalus. Fortschr Neurol Psycbaitr1995,63.248-51
16.
Aidi S., Elalaom-Faris M., Benabdeljlil M., Benomar A., Chaoui M., Chkili T.Akinetic mutism and progressive supranuclear palsy-like syndrome after the shunt of an obstructive hydrocephalus: successful treatment with bromocriptine 2 cases. Rev Neurol (Pans)2000;56. 380-3.
17.
Caner H., Altmors N., Benli S., Calisaneller T., Albayrak A.Akinetic mutism after fourth ventncle choroid plexus papilloma: treatment with a dopamine agonist. Surg Neurol1999;51:181-4. 18. Crismon ML, Childs A., Wilcox RE, Barrow N.The effect of bromocriptine on speech dysfunction m patients with diffuse brain injury (akinetic mutism. Clin Neuropharmacol1988,11.462-6. 19 Levm HS, Madison CF, Bailey CB, Meyers CA, Eisenberg HM, Gumto FCMutism after closed head injuryArch Neurol1983; 40.601-6
18.
Laplane D., Baulac M., Widlocher D., Dubois B.Pure psychic akinesia with bilateral lesions of basal ganglia. J Neurol Neurosurg Psychiatry1984,47:377-85
19.
Mega MS, Cohenour RCAkinetic mutism: disconnection of frontal-subcortical circuits. Neuropsychiatry Neuropsychol Behav Neurol1997,10.254-9.
20.
Ure J.,Faccio E., Videla H., et al Akinetic mutism: a report of three cases. Acta Neurol Scand1998,98.439-44.
21.
Botez MI, Barbeau A.Role of subcortical structures, and particularly of the thalamus, m the mechanisms of speech and language a review. Int J Neurol19718.300-20
22.
Castaigne P. , Lhermitte F., Buge A., Escourolle R., Hauw JJ, Lyon-Caen O.Paramedian thalamic and midbram infarct. clinical and neuropathological studyAnn Neurol, 1981 ;10.127-48.
23.
Guberman A. , Stuss D.The syndrome of bilateral paramedian thalamic infarction. Neurology1983;33.540-6.
24.
Katz DI, Alexander MP, Mandell AMDementia following strokes
25.
in the mesencephalon and diencephalonArch Neurol1987,441127-33
26.
Robles A., Aldrey JM, Rodriguez Fernandez RM, et al. Paramedian bithalamic infarct syndrome report of five new cases . Rev Neurol, 1995,23276-84.
27.
Segarra JMCerebral vascular disease and behavior, I. the syndrome of the mesencephalic artery (basilar artery bifurcationArch Neurol1970,22:408-18.
28.
Fallon JHTopographic organization of ascending dopaminergic projectionsAnn N Y Acad Sci1988;537:1-9.
29.
Ungerstedt U.Stereotaxic mapping of the monoamine pathways m the rat brain. Acta Physiol Scand Suppl1971,367.1-48 31. Lmdvall O., Bjorklund A., Moore RY, Stenevi U.Mesencephalic dopamine neurons projecting to neocortexBrain Res1974,81325-31.
30.
Nauta Whj, Feirtag M.Fundamental neuroanatomy. New YorkWH Freeman, 1986
31.
Baleydier C. , Mauguiere F The duality of the cingulate gyrus in monkey. Neuroanatomical study and functional hypothesis Bram1980,103:525-54
32.
Jurgens U.The efferent and afferent connections of the supplementary motor area. Bram Res1984;300.63-81
33.
Yakolev PL, Locke S.Limbic nuclei of the thalamus and connections of limbic cortex. Arch Neurol1961;5.364-400 36. Borggreve F., De Deyn PP, Marien P., Cras P., Dierckx RABilateral infarction in the anterior cerebral artery vascular territory due to an unusual anomaly of the circle of Willis (see commentsStroke1994;25:1279-81.
34.
Burns RS, Chiueh CC, Markey SP, Ebert MH, Jacobowitz DM, Kopm IJA primate model of parkinsonism: selective destruction of dopaminergic neurons in the pars compacta of the substantia nigra by N-methyl-4- phenyl-1,2,3,6-tetrahydropyridineProc Natl Acad Sci U S A1983;80.4546-50
35.
Horak FB, Anderson MEInfluence of globus pallidus on arm movements in monkeys, I effects of kainic acid-induced lesionsJ Neurophysiol1984,52:290-304.
36.
Iacono RP, Lonser RR, Mandybur G., Yamada S.Stimulation of the globus pallidus in Parkinson's disease. Br J Neurosurg1995,9.505-10
37.
Kuo JS, Carpenter MBOrganization of pallidothalamic projections in the rhesus monkey. J Comp Neurol1973,151201-36. 41. Daley DD, Love JGAkinetic mutismNeurology1958,8:238-42.
38.
Muller U., von Cramon DYThe therapeutic potential of bromocriptine in neuropsychological rehabilitation of patients with acquired brain damage. Prog NeuropsychopharmacolBiol Psychiatry1994,181103-20.
39.
Milhaud D., Bernardin G., Roger PM, Deloffre P., Corcelle P., Mattei M.Treatment of akinetic mutism with bromocriptine [Letter]. Presse Med1993,22.688.
40.
Barrett K.Treating organic abulia with bromocriptine and lisuride: four case studies . J Neurol Neurosurg Psychiatry1991,54. 718-21. 45. McDowell S., Whyte J., D'Esposito M.Differential effects of a dopaminergic agonist on prefrontal function in traumatic brain injury patients. Brain1998; 121.1155-64.
41.
Sawaguchi T. , Goldman-Rakic PSD1 dopamine receptors in prefrontal cortex: involvement in working memoryScience1991 , 251:947-50
