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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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