Frontal Lobe Syndromes
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The frontal lobe is the largest lobe in the brain, yet it is often not specifically evaluated in routine neurologic examinations. This may in part be due to the attention to detail and rigorous testing strategies required to probe frontal lobe functions. As successful completion of any cognitive task considered a frontal lobe function requires multiple brain regions both within and outside the frontal lobe, some authors prefer the term frontal systems disease. In any case, dysfunctions of the frontal lobe can give rise to relatively specific clinical syndromes. When a patient’s history suggests frontal lobe dysfunction, detailed neurobehavioral evaluation is necessary.
Traditional classification systems divide the frontal lobes into the precentral cortex (the strip immediately anterior to the central or Sylvian fissure) and prefrontal cortex (extending from the frontal poles to the precentral cortex and includes the frontal operculum), which is broken into: orbitofrontal cortex (including the orbitobasal or ventromedial and the inferior mesial regions), ventrolateral prefrontal cortex, dorsolateral prefrontal cortex, medial prefrontal cortex (containing the anterior cingulate gyrus, and prelimbic and infralimbic cortices), and the caudal prefrontal cortex (which includes the frontal eye fields). Each of these areas has widespread connectivity.
Given the unique connectivity between the frontal regions and deeper brain structures, lesions of these areas or their connections generate relatively distinctive clinical behaviors.
The dorsolateral frontal cortex is concerned with planning, strategy formation, and executive function. Patients with dorsolateral frontal lesions tend to have apathy, personality changes, abulia, and lack of ability to plan or to sequence actions or tasks. These patients have poor working memory for verbal information (if the left hemisphere is predominantly affected) or spatial information (if the right hemisphere bears the lesion brunt).
The frontal operculum contains the center for expression of language. Patients with left frontal operculum lesions may demonstrate Broca aphasia and defective verb retrieval, whereas patients with exclusively right opercular lesions tend to develop expressive aprosodia.
The orbitofrontal cortex is concerned with response inhibition. Patients with orbitofrontal lesions tend to have difficulty with disinhibition, emotional lability, and memory disorders. Patients with such acquired sociopathy, or pseudopsychopathic disorder, are said to have an orbital personality. Personality changes from orbital damage include impulsiveness, puerility, a jocular attitude, sexual disinhibition, and complete lack of concern for others.
Patients with lesions affecting the cingulate cortex typically develop akinetic mutism.
Patients with inferior mesial (basal forebrain) lesions tend to manifest anterograde and retrograde amnesia and confabulation.
Broca aphasia from a lesion in areas 44 and 45 on the left hemisphere leads to nonfluent speech, agrammatism, paraphasias, anomia, and poor repetition. Lesions anterior, superior, and deep to (but sparing) the Broca area produce abnormal syntax and grammar but repetition and automatic language are preserved. This disorder is known as transcortical motor aphasia (also called commissural dysphasia) and uninhibited echolalia is common. Memory disturbances only develop with lesion extension into the septal nucleus of the basal forebrain. Appreciation of verbal humor is most impaired in right frontal polar pathology.
The image below shows an MRI that is suggestive of frontotemporal dementia.
A detailed discussion of the pathophysiology of frontal lobe dysfunction is beyond the scope of this review and the reader is referred to 2 excellent reviews by Mesulam (2002) and Bonelli and Cummings (2007). [1, 2] As Mesulam has discussed, one way to think about the role of the frontal lobe is that it is the brain’s way of modifying and interposing constraints on basic reflexive behaviors. For example, taking food when one is hungry is reflexive. Nonetheless, most adults can inhibit this behavior until the context is appropriate. Most hungry diners waiting in line at a restaurant do not usually help themselves to food from the plates of diners who have already been served, but some patients with frontal lobe dysfunction cannot inhibit this response.
Unlike most animals, a human’s mental state is preoccupied a great deal with what has happened in the past or what may happen in the future. Parts of the frontal lobe are essential for this type of “time travel.” Indeed, good judgment requires evaluating the possible consequences of a variety of future activities and selecting the one with the most good consequences and the fewest bad consequences.
This frontal lobe-mediated responsibility of decision-making depends on the valuation of a choice, such as its costs, benefits, and probability of success, as well as the assessment of the outcome of a given choice, in order to adapt future behaviors appropriately. The anterior cingulate cortex is primarily responsible for selecting choices and evaluating the outcome of that selection to ensure adaptation to the environment. [1] The orbitofrontal cortex is responsible for changes in behavior in response to unexpected outcomes. [2] Poor judgment and inappropriately weighting of the value of past experiences may, as a result, occur with frontal lobe dysfunction.
Working memory involves a complex circuit that involves many brain regions, including the dorsolateral frontal cortex, thalamus, and parts of the temporal and parietal cortices. Working memory is defined as memory for a limited amount of information (such as a telephone number) that needs to be kept in consciousness for a few seconds (until the number is dialed) and then may be lost forever. Most patients are able to hold 6 or 7 digits in working memory. Patients with frontal lobe impairment may have decreased capacity in working memory (eg, shortened digit span) or difficulty manipulating information in working memory (eg, impaired reverse digit span test).
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Data are not available for the epidemiology of frontal lobe dysfunction as a clinical syndrome, but data are available concerning the incidence and prevalence of the major causes of syndromes of frontal lobe dysfunction. For specifics on these data, please refer to the following linked Medscape Reference articles. Common causes (see also Causes) include the following:
Intellectual disability
Traumatic brain injury (see Classification and Complications of Traumatic Brain Injury and Traumatic Brain Injury: Definition, Epidemiology, Pathophysiology
Brain tumors (see Brain Metastatis and EEG in Brain Tumors)
Degenerative dementias including Alzheimer disease, dementia with Lewy bodies, Parkinson-Plus Syndromes, and frontotemporal dementias
Cerebrovascular disease
Normal-pressure hydrocephalus and other hydrocephalic disorders
Psychiatric diseases such as schizophrenia and major depression
In addition, any neurologic or psychiatric disease that can affect the frontal lobe (eg, multiple sclerosis, CNS lupus) may be associated with frontal lobe dysfunction.
Frontal lobe dysfunction is associated with blood alcohol level and occurs during acute intoxication with many recreational drugs.
Traumatic brain injury is much more common in men than women both in the United States and worldwide. Gender predominance depends on the specific underlying neurologic disorder.
The relative likelihood of different causes of frontal lobe dysfunction is a function of patient age. In teenagers and young adults, the most common causes are intellectual disability, traumatic brain injury, and drug intoxication. In middle-aged patients, brain tumors, cerebrovascular disease, infections such as HIV, multiple sclerosis, and early onset degenerative dementias are common. In late life, cerebrovascular disease and degenerative dementias are predominant causes of frontal lobe dysfunction. The main degenerative dementias with frontal lobe predominance, frontotemporal lobar degenerations, together with Alzheimer disease, are the most common degenerative dementias in the pre-senile age (younger than 65 years).
Mesulam MM. The Human Frontal Lobes: Transcending the Default Mode through Continent Encoding. DT Stuss and RT Knight. Principles of Frontal Lobe Function. Oxford: 2002. 8-30.
Bonelli RM, Cummings JL. Frontal-subcortical circuitry and behavior. Dialogues Clin Neurosci. 2007. 9(2):141-51. [Medline].
Cruz-Oliver DM, Malmstrom TK, Allen CM, Tumosa N, Morley JE. The Veterans Affairs Saint Louis University Mental Status Exam (SLUMS Exam) and the Mini-Mental Status Exam as Predictors of Mortality and Institutionalization. J Nutr Health Aging. 2012. 16(7):636-41. [Medline].
Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: a Frontal Assessment Battery at bedside. Neurology. 2000 Dec 12. 55(11):1621-6. [Medline].
Nasreddine ZS, Phillips NA, Bedirian V, et al. The Montreal Cognitive Assessment, MoCA: a brief screening tool for mild cognitive impairment. J Am Geriatr Soc. 2005 Apr. 53(4):695-9. [Medline].
Kopp B, Rösser N, Tabeling S, Stürenburg HJ, de Haan B, Karnath HO, et al. Performance on the Frontal Assessment Battery is sensitive to frontal lobe damage in stroke patients. BMC Neurol. 2013 Nov 16. 13:179. [Medline]. [Full Text].
Munoz DP, Everling S. Look away: the anti-saccade task and the voluntary control of eye movement. Nat Rev Neurosci. Mar 5, 2004. 5:218-28. [Medline]. [Full Text].
Reitan RM. The relation of the trail making test to organic brain damage. J Consult Psychol. 1955 Oct. 19(5):393-4. [Medline].
Moll J, de Oliveira-Souza R, Moll FT, Bramati IE, Andreiuolo PA. The cerebral correlates of set-shifting: an fMRI study of the trail making test. Arq Neuropsiquiatr. 2002 Dec. 60(4):900-5. [Medline].
Pendleton MG, Heaton RK, Lehman RA, Hulihan D. Diagnostic utility of the Thurstone Word Fluency Test in neuropsychological evaluations. J Clin Neuropsychol. 1982 Dec. 4(4):307-17. [Medline].
Wu LJ, Sitburana O, Davidson A, Jankovic J. Applause sign in Parkinsonian disorders and Huntington’s disease. Mov Disord. 2008 Dec 15. 23(16):2307-11. [Medline].
Hama S, Yamashita H, Shigenobu M, Watanabe A, Kurisu K, Yamawaki S. Post-stroke affective or apathetic depression and lesion location: left frontal lobe and bilateral basal ganglia. Eur Arch Psychiatry Clin Neurosci. 2007 Apr. 257(3):149-52. [Medline].
Müller-Vahl KR, Kaufmann J, Grosskreutz J, Dengler R, Emrich HM, Peschel T. Prefrontal and anterior cingulate cortex abnormalities in Tourette Syndrome: evidence from voxel-based morphometry and magnetization transfer imaging. BMC Neurosci. 2009. 10:47. [Medline]. [Full Text].
Seltman RE, Matthews BR. Frontotemporal lobar degeneration: epidemiology, pathology, diagnosis and management. CNS Drugs. 2012 Oct 1. 26(10):841-70. [Medline].
Gorno-Tempini ML, Brambati SM, Ginex V, Ogar J, Dronkers NF, Marcone A. The logopenic/phonological variant of primary progressive aphasia. Neurology. 2008 Oct 14. 71(16):1227-34. [Medline].
Ismail Z, Nguyen MQ, Fischer CE, Schweizer TA, Mulsant BH. Neuroimaging of delusions in Alzheimer’s disease. Psychiatry Res. 2012 May 31. 202(2):89-95. [Medline].
Kerklaan BJ, van Berckel BN, Herholz K, Dols A, van der Flier WM, Scheltens P, et al. The added value of 18-fluorodeoxyglucose-positron emission tomography in the diagnosis of the behavioral variant of frontotemporal dementia. Am J Alzheimers Dis Other Demen. 2014 Nov. 29(7):607-13. [Medline].
Tsai RM, Boxer AL. Treatment of frontotemporal dementia. Curr Treat Options Neurol. 2014 Nov. 16(11):319. [Medline].
Baker M, Mackenzie IR, Pickering-Brown SM, et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature. 2006 Aug 24. 442(7105):916-9. [Medline].
Bech-Azeddine R, Hogh P, Juhler M, Gjerris F, Waldemar G. Idiopathic normal-pressure hydrocephalus: clinical comorbidity correlated with cerebral biopsy findings and outcome of cerebrospinal fluid shunting. J Neurol Neurosurg Psychiatry. 2007 Feb. 78(2):157-61. [Medline].
Benson DF, Miller BL. Frontal lobes, clinical and anatomic aspects. Feinberg TE, Farah MJ, eds. Behavioral Neurology and Neuropsychology. New York: McGraw Hill; 1997. 401-8.
Cairns NJ, Bigio EH, Mackenzie IR, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol. 2007 Jul. 114(1):5-22. [Medline]. [Full Text].
Damasio AR. The frontal lobes. Heilman KM, Valenstein E, eds. Clinical Neuropsychology. 3rd ed. New York: Oxford Univerisity Press; 1993. 409-60.
Kennerley SW, Wallis JD. Evaluating choices by single neurons in the frontal lobe: outcome value encoded across multiple decision variables. Eur J Neurosci. 2009 May. 29(10):2061-73. [Medline]. [Full Text].
Laisney M, Matuszewski V, Mezenge F, Belliard S, de la Sayette V, Eustache F. The underlying mechanisms of verbal fluency deficit in frontotemporal dementia and semantic dementia. J Neurol. 2009 Apr 14. [Medline].
Lezak MD. Neuropsychological Assessment. 3rd ed. New York: Oxford; 1995.
Luria AR. The Working Brain. An Introduction to Neuropsychology. Haig B, trans. New York: Basic Books; 1973.
Marmarou A, Bergsneider M, Klinge P, Relkin N, Black PM. The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus. Neurosurgery. 2005 Sep. 57(3 Suppl):S17-28; discussion ii-v. [Medline].
Mesulam MM. Principles of Behavioral Neurology. 2nd ed. New York: Oxford; 2000.
Nagaratnam N, Bou-Haidar P, Leung H. Confused and disturbed behavior in the elderly following silent frontal lobe infarction. Am J Alzheimers Dis Other Demen. 2003 Nov-Dec. 18(6):333-9. [Medline].
Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. 2006 Oct 6. 314(5796):130-3. [Medline].
Nyatsanza S, Shetty T, Gregory C, Lough S, Dawson K, Hodges JR. A study of stereotypic behaviours in Alzheimer’s disease and frontal and temporal variant frontotemporal dementia. J Neurol Neurosurg Psychiatry. 2003 Oct. 74(10):1398-402. [Medline].
Ondo WG, Chan LL, Levy JK. Vascular parkinsonism: clinical correlates predicting motor improvement after lumbar puncture. Mov Disord. 2002 Jan. 17(1):91-7. [Medline].
Stout JC, Wyman MF, Johnson SA, Peavy GM, Salmon DP. Frontal behavioral syndromes and functional status in probable Alzheimer disease. Am J Geriatr Psychiatry. 2003 Nov-Dec. 11(6):683-6. [Medline].
Takahashi YK, Roesch MR, Stalnaker TA, Haney RZ, Calu DJ, Taylor AR. The orbitofrontal cortex and ventral tegmental area are necessary for learning from unexpected outcomes. Neuron. 2009 Apr 30. 62(2):269-80. [Medline]. [Full Text].
Vecera SP, Rizzo M. Spatial attention: normal processes and their breakdown. Neurol Clin. 2003 Aug. 21(3):575-607. [Medline].
Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP Chief, Pediatric Neurology, Tulane Medical Center and Tulane-Lakeside Hospital; Pediatric Neurology Telemedicine/EEG, Women’s and Children’s Hospital and Lakeview Regional Medical Center; Associate Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Co-Director, Developmental Neurogenetics Center, Tulane University School of Medicine; Assistant Professor of Pediatrics, Neurology, and Psychiatry, Uniformed Services University of the Health Sciences
Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, Association of Military Surgeons of the US, Child Neurology Society, Southern Pediatric Neurology Society
Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Biomarin; Supernus<br/>Received income in an amount equal to or greater than $250 from: Biomarin; Supernus; American Board of Pediatrics.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Jasvinder Chawla, MD, MBA Chief of Neurology, Hines Veterans Affairs Hospital; Professor of Neurology, Loyola University Medical Center
Jasvinder Chawla, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association
Disclosure: Nothing to disclose.
Joseph Quinn, MD, MD Assistant Professor, Department of Neurology, Portland VA Medical Center, Oregon Health Sciences University
Joseph Quinn, MD, MD is a member of the following medical societies: American Academy of Neurology, Society for Neuroscience, Society for Pediatric Radiology
Disclosure: Nothing to disclose.
Alberto J Espay, MD, MSc Associate Professor, Director of Clinical Research, Gardner Family Center for Parkinson’s Disease and Movement Disorders, University of Cincinnati College of Medicine
Alberto J Espay, MD, MSc is a member of the following medical societies: American Academy of Neurology and Movement Disorders Society
Disclosure: Abbott Consulting fee Consulting; Chelsea therapeutics Consulting fee Consulting; Novartis Honoraria Speaking and teaching; TEVA Consulting fee Consulting; NIH Grant/research funds K23 Career Development Award; Eli Lilly Consulting fee Consulting; Great Lakes Neurotechnologies Other; Michael J Fox Foundation Grant/research funds Other; Lippincott Williams & Wilkins Royalty Book; American Academy of Neurology Honoraria Speaking and teaching
Frontal Lobe Syndromes
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