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    Strange Brains & Case Studies


    William Meil and I are compiling case studies to use for teaching (e.g. my Brain and Behavior class; To be fair, Bill did all of the work on the case studies; see his article "The Use of Case Studies in Teaching Undergraduate Neuroscience").  Below, we list strange things that might happen to your brain and, when possible, case studies describing such atypical functioning.  Often Wikipedia descriptions and links have been provided as an introduction to these strange brains and reference to popular science books are provided for case studies.  Please note that neither should be considered the paragon of truth and both should be read with some, if not heaps of skepticism.  That said, case studies are a useful method of explication because, among other reasons, they add a humanistic element and provide a flavor for how incredibly interesting and strange the brain is that is often lacking in (dry) neuroscience textbooks.  

    If you would like to add to the following list, please do so via the "Comments?" page.    


    Acalculia


    Wikipedia Description: "An acquired impairment in which patients have difficulty performing simple mathematical tasks, such as adding, subtracting, multiplying and even simply stating which of two numbers is larger. Acalculia is distinguished from dyscalculia in that acalculia is acquired late in life due to neurological injury such as stroke, while dyscalculia is a specific developmental disorder first observed during the acquisition of mathematical knowledge."

    Further links and references:



    Achromatopsia


    Wikipedia Description: "Typically refers to an autosomal recessive congenital color vision disorder, the inability to perceive color AND to achieve satisfactory visual acuity at high light levels (typically exterior daylight)."

    Case Study:
        Title: The Case of the Colorblind Painter
        Notes: Tracks influence on case’s life and art
        Reference: Sacks, O. (1995). An anthropologist on mars. New York, NY: Vintage Books. 

    Further links and references:



    Acromegaly


    Wikipedia Description: "syndrome that results when the pituitary gland produces excess growth hormone (hGH) after epiphyseal plate closure at puberty."

    Case Study:
        Title: Giants Among Men
        Notes: Parallels Roman emperor and basketball player with excessive growth hormone release
        Reference: Klawans, H. L. (1988). Toscanini’s fumble: And other tales of clinical neurology. Chicago, IL: Contemporary Books. 

    Case Study:
        Title: Primo Carnera: The Bigger They Are
        Notes: Story of a boxer with excessive release of growth hormone

    Further links and references:



    Agrammatism


    Wikipedia Description: "A form of expressive aphasia that refers to the inability to speak in a grammatically correct fashion."

    Further links and references:



    Akinetopsia or Motion Blindness


    Wikipedia Description: "An extremely rare neuropsychological disorder in which a patient cannot perceive motion in his/her visual field, despite being able to see stationary objects without issue." 

    Further links and references:



    Alexia or Word Blindness

    AKA: Text Blindness or Visual Aphasia

    Wikipedia Description: "Damage to the brain causes a patient to lose the ability to read." 

    Further links and references:



    Alexithymia


    Wikipedia Description: "A state of deficiency in understanding, processing, or describing emotions."

    Further links and references:



    Alice in Wonderland syndrome

    AKA: Todd's syndrome

    Wikipedia Description: "A disorienting neurological condition which affects human perception. Sufferers may experience micropsia, macropsia, and/or size distortion of other sensory modalities."

    Links, references, and videos:



    Alien Hand Syndrome

    AKA: Anarchic Hand or Dr. Strangelove syndrome

    Wikipedia Description: "An unusual neurological disorder in which one of the sufferer's hands seem to take on a mind of its own."

    Links and references:




    Allochiria


    Wikipedia Description: "A neurological disorder in which the patient responds to stimuli presented to one side of their body as if the stimuli had been presented at the opposite side."

    Links and references:



    Amusia


    Wikipedia Description: "A musical disorder that appears mainly as a defect in processing pitch, but it also encompasses musical memory and recognition."

    Case Study:
        Title: 
    Broca’s Amusica
        Notes: 
    Loss of musical abilities following a stroke; Describes a woman who selectively looses her ability to play the oboe.  
        
    Reference: Klawans, H. L. (1988). Toscanini’s fumble: And other tales of clinical neurology. Chicago, IL: Contemporary Books. 

    Links and references:



    Anomia


    Wikipedia Description: "A severe problem with recalling words or names."

    Links and references:



    Anosognosia


    Wikipedia Description: "condition in which a person who suffers disability seems unaware of or denies the existence of his or her disability. This may include unawareness of quite dramatic impairments, such as blindness or paralysis"

    Case Study:
        Title: The Sound of One Hand Clapping
        Notes: Denial syndrome
        Reference: Ramachandran, V.S., and Blakeslee, S. (1998). Phantoms in the brain: probing the mysteries of the human mind. New York, NY: William Morrow and Company, Inc.  

    Further links and references:


    Antero- and Retrograde Amnesia


    Categories: Memory

    Wikipedia Description: "Anterograde amnesia is a loss of the ability to create new memories after the event that caused the amnesia, leading to a partial or complete inability to recall the recent past, while long term memories from before the event remain intact." and "Retrograde amnesia is a form of amnesia where someone is unable to recall events that occurred before the development of the amnesia."

    Case Study:
        Title: The Last Hippie
        Notes: Addresses several other issues: pituitary tumors, religion, anosognosia. May interest fans of 60’s rock music.
        Reference: Sacks, O. (1995). An anthropologist on mars. New York, NY: Vintage Books.

    Case Study:
        Title: Marooned in the Moment: H.M. a Case of Global Amnesia
        Notes: Case of Henry Gustav Molaison (i.e., H.M.), who was "a memory-impaired patient who was widely studied from the late 1950s until his death. His        case played a very important role in the development of theories that explain the link between brain function and memory, and in the development of cogni    tive neuropsychology, a branch of psychology that aims to understand how the structure and function of the brain relates to specific psychological proces    ses." This case study has a qualitative twist.
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press.


    Links and references:



    Anton–Babinski Syndrome


    Wikipedia Description: "People who suffer from it are "cortically blind", but affirm, often quite adamantly and in the face of clear evidence of their blindness, that they are capable of seeing."

    Links and references:



    Aphasia


    Wikipedia Description: "An acquired language disorder in which there is an impairment of any language modality."

    Case Study:
        Title: Defending the Cavewoman (Chapter 1)
        Notes: 
    Language acquisition (from the perspective of 
    early abuse) 
    with an eye on evolution.  
        Reference: Klawans, H. L. (2000). Strange behavior: Tales of evolutionary neurology. New York, NY: W.W. Norton & Company.

    Links and references:



    Aprosodia


    Wikipedia Description: "A neurological condition characterized by the inability of a person to properly convey and/or interpret emotional prosody."

    Links and references:



    Autism Spectrum Disorder and Aspergers


    Wikipedia Description: "The autism spectrum disorders is a spectrum of psychological conditions characterized by widespread abnormalities of social interactions and communication, as well as severely restricted interests and highly repetitive behavior." and "Asperger's syndrome is an autism spectrum disorder that is characterized by significant difficulties in social interaction, along with restricted and repetitive patterns of behavior and interests. It differs from other autism spectrum disorders by its relative preservation of linguistic and cognitive development."

    Case Study:
        Title: 
    An Anthropologist on Mars
        Notes: 
    Extremely insightful look at autism from within
        Reference: 
    Sacks, O. (1995). An anthropologist on mars. New York, NY: Vintage Books.

    Case Study:
        Title: 
    Inside the Mind of a Savant
        Notes: 
    Case of Kim Peek, the inspiration for the movie “Rain Man”
        Reference: 
    Treffert and Christensen (2006)

    Case Study:
        Title: Prodigies
        Notes: 
    Chronicles an artistic savant
        Reference: 
    Sacks, O. (1995). An anthropologist on mars. New York, NY: Vintage Books.

    Case Study:
        Title: 

    The Surfing Savant

        Notes: Compelling story of how Pro surfer’s Asperger’s symptoms both help and hinder him.  
        Reference: 

    Solotaroff, P (2010) The Surfing Savant. Rolling Stone. Issue 1102, April 15, 2010


    Links and references: 



    Autotopagnosia


    Wikipedia Description: "Is agnosia that affects the sense of posture; characterized by an inability to localize and orient different parts of the body."

    Case Study: 
        Title: A Body in the Mind: A Case of Autotopagnosia
        Notes: 
    Gerstmann's Syndrome; 
    Covers a variety of often related perceptual deficits
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Links and references:



    Balint's Syndrome


    Wikipedia Description: "Characterized by optic ataxia (incoordination of hand and eye movement), oculomotor apraxia (the inability to voluntarily guide eye movements/ change to a new location of visual fixation), and simultanagnosia (the inability to perceive more than one object at a time, even when in the same place)."

    Links and references:



    Blindsight


    Wikipedia Description: "A phenomenon in which people who are perceptually blind in a certain area of their visual field demonstrate some response to visual stimuli."

    Links and references:



    Capgras delusion


    Wikipedia Description: "disorder in which a person holds a delusion that a friend, spouse, parent or other close family member has been replaced by an identical-looking impostor. The Capgras delusion is classed as a delusional misidentification syndrome, a class of delusional beliefs that involves the misidentification of people, places, or objects. It can occur in acute, transient, or chronic forms." 

    Case Study:
        Title: 
    The Unbearable Likeness of Being
        Notes: 
    Provides insight into the link between visual recognition and emotion
        Reference: Ramachandran, V.S., and Blakeslee, S. (1998). Phantoms in the brain: probing the mysteries of the human mind. New York, NY: William Morrow and Company, Inc.  

    Further links and references:



    Cerebral Achromatopsia


    Wikipedia Description: "A type of color-blindness that is caused by damage to the cerebral cortex of the brain, rather than abnormalities in the cells of the eye's retina."

    Links and references:



    Charles Bonnet Syndrome


    Wikipedia Description: "A condition that causes patients with visual loss to have complex visual hallucinations."

    Case Study:
        Title: 
    The Secret Life of James Thurber
        Notes: 
    Visual hallucinations in the blind
        Reference: Ramachandran, V.S., and Blakeslee, S. (1998). Phantoms in the brain: probing the mysteries of the human mind. New York, NY: William Morrow and Company, Inc.  

    Further links and references:



    Conduction Aphasia

    AKA: Associative Aphasia

    Wikipedia Description: "An acquired language disorder, it is characterized by intact auditory comprehension, fluent (yet paraphasic) speech production, but poor speech repetition."

    Links and references:



    Cotard Delusion 

    AKA: Cotard's Syndrome or Walking Corpse Syndrome

    Wikipedia Description: "A rare neuropsychiatric disorder in which people hold a delusional belief that they are dead (either figuratively or literally), do not exist, are putrefying, or have lost their blood or internal organs. Rarely, it can include delusions of immortality."

    Links, references, and videos:



    Discalculia


    Wikipedia Description: "A specific [developmental] learning disability or difficulty involving... difficulty in learning or comprehending mathematics."

    Links and references:



    Dysprosody


    Wikipedia Description: "Also known as pseudo-foreign dialect syndrome, refers to a disorder in which one or more of these prosodic functions are either compromised or eliminated completely."

    Links and references:



    Expressive or Broca's Aphasia


    Wikipedia Description: "Is characterized by the loss of the ability to produce language (spoken or written)."

    Case Study:
        Title: 
    The Breakdown of Language: Case Studies of Aphasia
        Notes: Two cases illustrate Broca’s and Wernicke’s aphasia, extensive background information
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Links and references:



    Foreign Accent Syndrome


    Wikipedia Description: "To the untrained ear, those with the syndrome sound as though they speak their native languages with a foreign accent."

    Links and references:



    Fregoli delusion


    Wikipedia Description: "A rare disorder in which a person holds a delusional belief that different people are in fact a single person who changes appearance or is in disguise." 

    Links and references:



    Gaze-evoked Tinnitus


    Description: "Hear a ringing sound when looking to the left (or right)."

    Links and references:
        Coad ML, Lockwood A, Salvi R, Burkard R. (2001). Characteristics of patients with gaze-evoked tinnitus. Otol Neurotol. 22(5):650-4. 



    Hemiplegia


    Wikipedia Description: "A condition in which the limbs on one side of the body have severe weakness. Hemiplegia is more severe than hemiparesis, wherein one half of the body has less marked weakness.[1]Hemiplegia may be congenital or acquired from an illness or stroke."

    Case Study:
        Title: My Mother’s Best Friend (Chapter 1)
        Notes: Anosognosia; Also touches on strokes and aphasia.
        Reference: 
    Klawans, H. L. (1990). Newton’s madness: Further tales of clinical neurology. New York, NY: Harper and Row Publishers.
     

    Case Study:
        Title: The Man Who Fell Out of Bed
        Notes: Anosognosia; Very short, lacking recent background information
        Reference: 
    Sacks, O. (1985). The man who mistook his wife for a hat and other clinical tales. New York, NY: Harper and Row Publishers.
     

    Links and references:



    Hemispatial Neglect


    Wikipedia Description: "A condition in which, after damage to one hemisphere of the brain, a deficit in attention to and awareness of one side of space is observed."

    Case Study:
        Title: Out of mind, out of sight: a case of hemineglect
        Notes: Covers topic in great depth.
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Case Study:
        Title: Through the Looking Glass
        Reference: 
    and Blakeslee, S. (1998). Phantoms in the brain: probing the mysteries of the human mind. New York, NY: William Morrow and Company, Inc

    Links and references:




    Huntington's disease


    Wikipedia Description: "A progressive neurodegenerative genetic disorder, which affects muscle coordination and leads to cognitive decline and dementia. It typically becomes noticeable in middle age. HD is the most common genetic cause of abnormal involuntary writhing movements called chorea and is much more common in people of Western European descent than in those from Asia or Africa."

    Case Study:
        Title: Anticipation
        Notes: Lacking most recent information
        Reference: (2000). Strange behavior: Tales of evolutionary neurology. New York, NY: W.W. Norton & Company. 

    Case Study:
        Title: Huntington’s Disease: A Family Challenged
        Notes: Chronicles the struggle of a family with Huntington’s disease, extensive background information.  
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Case Study:
        Title: Spontaneous Generation
        Notes: Genetics and Huntington’s disease, lacking most recent information.
        Reference: (1988). Toscanini’s fumble: And other tales of clinical neurology. Chicago, IL: Contemporary Books. 

    Links and references:



    Hyperlexia


    Wikipedia Description: "It can be viewed as a superability in which word recognition ability goes far above expected levels of skill."

    Links, references, and videos:



    Ideomotor Apraxia


    Wikipedia Description: "A neurological disorder characterized by the inability to correctly imitate hand gestures and voluntarily pantomime tool use, e.g. pretend to brush one's hair."

    Links and references:



    Jumping Frenchmen of Maine


    Wikipedia Description: "It results in an exaggerated "startle" reflex.. for instance, individuals who would obey any command given suddenly, even if it meant striking a loved one, and repeat back unfamiliar or foreign phrases uncontrollably."

    Links and references:



    Korsakoff's syndrome


    Category: Memory

    Wikipedia Description: "a neurological disorder caused by the lack of thiamine (vitamin B1) in the brain... There are six major symptoms of Korsakoff's syndrome: 1) anterograde amnesia; 2) retrograde amnesia; 3) confabulation, that is, invented memories which are then taken as true due to gaps in memory sometimes associated with blackouts; 4) meager content in conversation; 5) lack of insight; and 6) apathy."

    Case Study:
        Title: 
    The Lost Mariner
        Notes: 
    Lacking recent background information
        Reference: 
    Sacks, O. (1985). The man who mistook his wife for a hat and other clinical tales. New York, NY: Harper and Row Publishers. 

    Case Study:
        Title: 
    Morbid Obesity
        Notes: 
    Case of Korsakoff’s syndrome after gastric bypass surgery.
        Reference: 
    Klawans, H. L. (1990). Newton’s madness: Further tales of clinical neurology. New York, NY: Harper and Row Publishers.

    See also: Anterograde and Retrograde Amnesia

    Links and references:




    King-Kopetzky Syndrome


    Wikipedia Description: "Difficulty in hearing speech in the presence of background noise. This is in conjunction with the finding of normal hearing test results."

    Links and references:


    Kluver-Bucy Syndrome


    Wikipedia Description: "Symptoms..  included are... Docility; Dietary changes; Hyperorality; Hypersexuality; Visual agnosia."

    Links and references:



    Macropsia

    AKA: Megalopia

    Wikipedia Description: "A neurological condition affecting human visual perception, in which objects within an affected section of the visual field appear larger than normal, causing the subject to feel smaller."

    Links and references:


    Micropsia


    Wikipedia Description: "A condition affecting human visual perception in which objects are perceived to be smaller than they actually are."

    Links and references:



    Multiple sclerosis or MS


    Wikipedia Description: "is a disease in which the fatty myelin sheaths around the axons of the brain and spinal cord are damaged, leading to demyelination and scarring as well as a broad spectrum of signs and symptoms... The person with MS can suffer almost any neurological symptom or sign, including changes in sensation (hypoesthesia and paraesthesia), muscle weakness, muscle spasms, or difficulty in moving; difficulties with coordination and balance (ataxia); problems in speech (dysarthria) or swallowing (dysphagia), visual problems (nystagmusoptic neuritis, or diplopia), fatigue, acute or chronic pain, and bladder and bowel difficulties. Cognitive impairment of varying degrees and emotional symptoms of depression or unstable mood are also common.[1] Uhthoff's phenomenon, an exacerbation of extant symptoms due to an exposure to higher than usual ambient temperatures, and Lhermitte's sign, an electrical sensation that runs down the back when bending the neck, are particularly characteristic of MS although not specific." 

    Case Study:
        Title: The Bobbsey Twins Take Neurology
        Notes: Covers its motor and perceptual nature
        Reference: Klawans, H. L. (1990). Newton’s madness: Further tales of clinical neurology. New York, NY: Harper and Row Publishers. 

    Case Study:
        Title: Tomorrow is Another Day: Living with Multiple Sclerosis
        Notes: Describes case of a woman and illustrates the variable nature of this disorder
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Links and references:



    Paraphasia


    Wikipedia Description: "A feature of aphasia in which one loses the ability of speaking correctly, substitutes one word for another, and changes words and sentences in an inappropriate way."

    Links and references:



    Parkinson's disease


    Wikipedia Description: "A degenerative disorder of the central nervous system that often impairs the sufferer's motor skills, speech, and other functions."

    Case Study:
        Title: Mind Over Matter: Coping with Parkinson’s Disease
        Notes: Describes case of a medical doctor with Parkinson’s disease, pallidotomy, extensive background information. 
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Case Study:
        Title: The Lizard
        Notes: Covers treatment with L-dopa and adrenal transplants. 
        Reference: Klawans, H. L. (1990). Newton’s madness: Further tales of clinical neurology. New York, NY: Harper and Row Publishers. 

    Links and references:
        Wikipedia: http://en.wikipedia.org/wiki/Parkinsons



    Phantom Limb


    Wikipedia Description: "Is the sensation that an amputated or missing limb (even an organ, like the appendix) is still attached to the body and is moving appropriately with other body parts.[1][2][3] Approximately 60 to 80% of individuals with an amputation experience phantom sensations in their amputated limb, and the majority of the sensations are painful.[4] Phantom sensations may also occur after the removal of body parts other than the limbs, e.g. after amputation of the breast, extraction of a tooth (phantom tooth pain) or removal of an eye (phantom eye syndrome). The missing limb often feels shorter and may feel as if it is in a distorted and painful position."

    Case Study:
        Title: 
    The Phantom Within (Chapter 1); Knowing Where to Scratch 
    (Chapter 2) and
    ; Chasing the Phantom (Chaper 3)
        Notes: 
    Tale tracing phantom limbs to neural plasticity.  
        Reference: Ramachandran, V.S., and Blakeslee, S. (1998). Phantoms in the brain: probing the mysteries of the human mind. New York, NY: William Morrow and Company, Inc.  

    Further links and references:



    Pragmatic language impairment


    Wikipedia Description: "People with these impairments have special challenges with the semantic aspect of language (the meaning of what is being said) and the pragmatics of language (using language appropriately in social situations)."

    Links and references:



    Prosopagnosia


    Wikipedia Description: "A disorder of face perception where the ability to recognize faces is impaired, while the ability to recognize other objects may be relatively intact."

    Links and references:



    Pure Word Deafness


    Wikipedia Description: "Inability to comprehend the meaning of speech, but (in most cases) still being able to hear, speak, read, and write."

    Links and references:



    Receptive or Wernicke's Aphasia


    Wikipedia Description: "Speech is preserved, but language content is incorrect."

    Case Study:
        Title: 
    The Breakdown of Language: Case Studies of Aphasia
        Notes: Two cases illustrate Broca’s and Wernicke’s aphasia, extensive background information
        Reference: Ogden, J. A. (2005). Fractured minds. New York, NY: Oxford University Press. 

    Links and references:



    Reduplicative Paramnesia


    Wikipedia Description: "Is the delusional belief that a place or location has been duplicated, existing in two or more places simultaneously, or that it has been 'relocated' to another site."

    Links and references:



    Schizophrenia


    Wikipedia Description: "A severe mental illness characterized by a disintegration of the process of thinking, of contact with reality, and of emotional responsiveness.  It most commonly manifests as auditory hallucinations, paranoid or bizarre delusions, or disorganized speech and thinking with significant social or occupational dysfunction."

    Links and references:



    Somatoparaphrenia


    Wikipedia Description: "A type of monothematic delusion where one denies ownership of a limb or an entire side of one's body."

    Links and references:



    Synesthesia


    Wikipedia Description: "A neurologically-based condition in which stimulation of one sensory or cognitive pathway leads to automatic, involuntary experiences in a second sensory or cognitive pathway."

    Case Study:
        Title: The Man Who Tasted Shapes
        Notes: This book contains many case studies on synesthesia
        Reference: Cytowic, R. E. (2003). The man who tasted shapes. Cambridge, MA: MIT Press. 

    Links and references:




    Tourette's Syndrome


    Category: Motor

    Wikipedia Description: "Characterized by multiple physical (motor) tics and at least one vocal (phonic) tic."

    Case Study: 
        Title: A Surgeon’s Life
        Notes: Great success in the face of adversity.
        Reference: Sacks, O. (1995). An anthropologist on mars. New York, NY: Vintage Books. 
    Case Study: 
        Title: Just right: Mahmoud Abdul-Rauf
        Notes: Describes case of professional basketball player with the disorder.  
        Reference: Klawans, H. L. (1996). Why Michael couldn’t hit: and other tales of the neurology of sports. New York, NY: W.H. Freeman. 
    Case Study:
        Title: 
    Witty Ticcy Ray
        Notes: Lacking recent background information
        Reference: Sacks, O. (1985). The man who mistook his wife for a hat and other clinical tales. New York, NY: Harper and Row Publishers. 

    Links and references:


    Visual Agnosia

    Wikipedia Description: "The inability of the brain to make sense of or make use of some part of otherwise normal visual stimulus and is typified by the inability to recognize familiar objects or faces."

    Links and references:



    Williams Syndrome


    Wikipedia Description: "A distinctive, "elfin" facial appearance, along with a low nasal bridge; an unusually cheerful demeanor and ease with strangers; developmental delay coupled with unusual (for persons who are diagnosed as developmentally delayed) language skills; and cardiovascular problems, such as supravalvular aortic stenosis and transient hypercalcaemia."

    Links and references:


    Wilson's Disease


    Wikipedia Description: "An autosomal recessive genetic disorder in which copper accumulates in tissues... about half the patients with Wilson's have neurological or psychiatric problems. Most patients initially have mild cognitive deterioration and clumsiness, as well as changes in behavior. Specific neurological symptoms then follow, often in the form of parkinsonism (increased rigidity and slowing of routine movements) with or without a typical hand tremor, masked facial expressions, slurred speech, ataxia (lack of coordination) or dystonia (twisting and repetitive movements of part of the body)."

    Case Study: 
        Title: Still Smiling (Chapter 2)
        Notes: Genetic disorder - copper metabolism.  
        Reference: Klawans, H. L. (1990). Newton’s madness: Further tales of clinical neurology. New York, NY: Harper and Row Publishers. 

    Links and references:

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