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Lessons from the Works of Oliver Sacks FUNCTIONAL CORRELATES OF GYRAL ANATOMY S Wright | RT Fitzgerald eEdE-19 Disclosures I have no commercial interests to disclose. Purpose and Methods Purpose: This presentation will review specialized areas of the cerebral cortex and their associated functions and illustrate the importance of familiarity with this topic. Methods: We will draw from the prolific works of Oliver Sacks to show clinical correlation of anatomy and function. Lastly, image-based cases will highlight real-life scenarios. How is this helpful? • Radiologists can feel removed from what “abnormal signal” in a brain can mean for a patient. • Being familiar with how lesions present can help with communication between radiologists and clinical colleagues. • With the aid of a focused clinical history and neurological examination, specific areas can be scrutinized more closely, increasing our sensitivity for subtle findings. Oliver Sacks • British Neurologist 1933-2015 • Prolific writer of lay and academic neurologic casebased literature, much of which focuses on correlation of clinical presentation with lesion localization • “Poet laureate of contemporary medicine.” – The New York Times The Cerebrum • The cerebrum arises from the embryonic telencephalon • This is the “newest” portion of the central nervous system, in terms of evolutionary development. Case courtesy of Dr Jeremy Jones, Radiopaedia.org, rID: 36277 Cerebral Cortex Hagmann P, et al. Highly specialized infoldings of the cerebrum correlate with physical, emotional, and mental functioning. Frontal Lobe Gyri Lateral surface: Superior, middle and inferior frontal gyri, precentral gyrus, opercular gyrus, and premotor area Orbital surface: Orbital (lateral, anterior, posterior, medial) gyri, gyrus rectus Medial surface: Paracentral lobule, cingulate gyrus S. Lamb. Rice University Temporal Lobe Gyri Lateral surface: Superior, middle, and inferior temporal gyri Basal surface: Occipitotemporal gyrus S. Lamb. Rice University Medial Temporal Lobe Takahashi T, et al. 2006 The medial temporal lobe is part of the limbic system and includes the uncus, parahippocampal gyrus, and fusiform gyrus. Parietal Lobe Gyri Lateral Surface: Postcentral gyrus, superior parietal lobule, inferior parietal lobule (supramarginal and angular gyri) Medial surface: Paracentral lobule, precuneus S. Lamb. Rice University. Occipital Lobe Gyri Lateral surface: Superior, middle, and inferior occipital gyri Medial surface: Cuneus, lingual gyrus Medial Cortical Surface Frontal Lobe Functions General functions of the frontal lobe include executive decision-making and social behavior. More specialized regions include the precentral gyrus, which controls contralateral motor function and Broca’s area which controls speech motor activity in the dominant hemisphere. Case from Oliver Sacks “Mrs. B.”, a research chemist, presented with a rapid personality change. She became “funny,” “facetious,” “impulsive-and superficial.” A friend says, “She no longer seems to care about anything at all.” The initial working diagnosis was hypomania, but search for an organic cause was performed. Excerpt from “Yes, Father-Sister” in The Man Who Mistook His Wife for a Hat “Her face bore no deeper expression whatever. Her world had been voided of feeling and meaning. Nothing any longer felt ‘real’ (or ‘unreal’). Everything was now ‘equivalent’ or ‘equal’-the whole world reduced to a facetious insignificance.” Diagnosis Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 8861 “At first it was thought that she might be hypomanic, but she turned out to have a cerebral tumour. At craniotomy there was found, not a meningioma as had been hoped, but a huge carcinoma involving the orbitofrontal aspects of both frontal lobes.” T2W image of esthesioneuroblastoma infiltrating into the orbitofrontal cerebrum. Temporal Lobe Functions The lateral temporal lobe includes, among others, specialized areas for auditory processing, as well as Wernicke’s area for comprehension of spoken language. The medial temporal lobe contains many structures of the limbic system, which controls emotion, behavior, olfactory processing, and memory. Case from Oliver Sacks “Mrs. O’C”, a nursing home resident, complained of sudden onset of persistently playing music, which she originally thought was due to a record player being turned on at the residence. Consultation with ENT and psychiatry was obtained before neurology consultation with Dr. Sacks. Excerpt from “Reminiscence” in The Man Who Mistook His Wife for a Hat “One night, in January 1979, she dreamt vividly, nostalgically, of her childhood in Ireland, and especially of the songs they danced to and sang. When she woke up, the music was still going, very loud and clear.” Diagnosis Di Pietro M, et al. “I obtained a brainscan, and this showed that she had indeed had a small thrombosis…in part of her right temporal lobe. The sudden onset of Irish songs in the night, the sudden activation of musical memory-traces in the cortex, were, apparently, the consequence of a stroke, and as it resolved, so the songs ‘resolved’ too.” Images demonstrate infarct involving the superior temporal gyrus. This patient had musical deficits, as opposed to positive musical symptoms. Parietal Lobe Functions Specialized functions of the parietal lobe include sensation and spatial perception. Example of a drawing of a clock in patient with hemineglect syndrome from a unilateral parietal lesion Know the Central Sulcus! • It divides the precentral gyrus of the frontal lobe from the postcentral gyrus of the parietal lobe • These are known as the primary motor and sensory strips, respectively • Localized lesions along these gyri present in very predictable manners. Case courtesy of A.Prof Frank Gaillard, Radiopaedia.org, rID: 33834 Case from Oliver Sacks A patient on the neurology ward for a pre-existing condition awoke without sensation of one of his legs; further, the patient did not recognize the leg as belonging to him. Excerpt from “The Leg” “I suddenly remembered a patient on the Neurology Wards 15 years before, who had woken from sleep, one Christmas Day, to find ‘a strange leg’ in his bed. There was a Christmas party going on, and the only ‘explanation’ he could imagine was that one of the nurses, as a joke, had filched a leg from the Anatomy Room, and slipped it under the covers as he slept. Amused, and disgusted, he threw ‘the damn thing’ out of the bed, but then found, to his inexpressible amazement and horror, that he came out with it, that ‘the damn thing’ was attached to him.” Diagnosis OpenStax College - Anatomy & Physiology, Connexions Web site. “It turned out that he had a tumour of the right parietal lobe of his brain, which had haemorrhaged while he was snoozing on Christmas Day, obliterating the ‘leg area’ of the brain, and thus obliterating any sense of his own leg, so that, when he ‘discovered’ it, it was not his, but ‘a damn thing’.” Sensory Homunculus Homunculus Different parts of the body are located at very specific locations of the motor and sensory strips. The homunculus is a visual representation of body parts mapped onto the structures of the correlating cortex responsible for their sensory or motor functions. Occipital Lobe Functions Dr. Nisreen Abo-Elmaaty The occipital lobe receives the input from the optic system and is responsible for visual processing. Oliver Sacks Case “Rosalie”, a 90 year old woman, completely blind from acquired retinal damage, presented with vivid and complex visual hallucinatory scenes which persist for long periods of time. The patient was seen by a psychiatrist who found no mental disease. A neurology consultation was requested, and she was seen by Dr. Sacks. Excerpt from Hallucinations “I observed with Rosalie (as with many other patients) that while she was hallucinating, her eyes were open, and even though she could see nothing, her eyes moved here and there, as if looking at an actual scene.” Diagnosis National Eye Institute of the NIH “I explained to her that hallucinations, strangely, are not uncommon in those with blindness or impaired sight, and that these visions are not “psychiatric” but a reaction of the brain to the loss of eyesight. She had a condition called Charles Bonnet syndrome.” CBS patients have been shown to have aberrant occipital lobe uptake on FDG-PET, and it is theorized this is due to deafferentiation, or loss of input, to the visual cortex of the occipital lobe. Fundoscopic image demonstrating changes of macular degeneration Case 1 The patient is a 60 year old male with history of chest wall melanoma, negative margins at resection. A year later, patient presents with acute left-sided weakness. Imaging was obtained at presentation. SWI FLAIR Case 1 The SWI and FLAIR images show a large, hemorrhagic melanoma lesion in the high right frontal lobe with a large amount of adjacent edema. The mass effect compresses the patient’s motor strip, thereby resulting in the left-sided motor deficits seen on examination. While not a small or subtle lesion, this case shows good clinical and imaging correlation. Case 2 A 32 year old, otherwise healthy female presents with persistent auditory hallucinations. Schizophrenia is the working diagnosis, but a search for an organic cause is performed. FLAIR T1 +C Case 2 The preceding axial and coronal images show a FLAIR hyperintense, non-enhancing mass in the superior temporal gyrus, which contains the primary auditory cortex. The low-grade glioma was resected, and the hallucinations resolved. Case 3 A 45 year old male presents for evaluation of right inferior quadrantanopia. Further history reveals recent ataxia and cognitive decline. DWI Case 3 The preceding axial DWI slice shows restricted diffusion involving the left occipital cortex. With other testing, the diagnosis of Creutzfeldt-Jakob disease was made. The location of the cortical signal abnormality correlates with the patient’s isolated right visual field deficit. The diagnosis of CJD is also consistent with other components of the patient’s presentation. Summary Radiologists are good at thoroughly reviewing images, but knowing where to look can narrow our focus and increase our sensitivity for finding subtle abnormalities that may be clinically significant. Don’t hesitate to do a little digging and to bend the ear of our clinical neurological colleagues. Like Dr. Sacks, they often have a keen perception and know what they are expecting to see before the study is performed. References Sacks, O. (1985). The man who mistook his wife for a hat and other clinical tales. New York: Summit Books. Sacks, O. (2012). Hallucinations. New York: Random House. Sacks, O. (1982). The leg. London Review of Books, 11(4), 3-5. Afifi, A. K., & Bergman, R. A. (1998). Functional neuroanatomy: Text and atlas. New York: McGraw-Hill, Health Professions Division. Di Pietro, M., Laganaro, M., Leemann, B., Schnider, A. “Receptive amusia: temporal auditory processing deficit in a professional musician following a left temporo-parietal lesion” Neuropsychologi 42 (2004): 868-877 April 2016.