30 December 2008

Foreign Accents, Alien Hands and Other Medical Oddities

F. Scott Fitzgerald invented the reverse-aging phenomenon at the heart of "The Curious Case of Benjamin Button," which opened at movie theaters this week. But there are plenty of unusual ailments that are quite real.

Here are just a few of them:

Foreign Accent Syndrome. Seemingly out of the blue, sufferers start speaking with what sounds like a foreign accent -- often of a country they've never visited. In one noted case, a Norwegian woman developed a German accent after being hit by bomb shrapnel in 1941. Neighbors suspected she was a spy. Several dozen cases have been documented world-wide. Victims are often accused of faking the condition, but it generally follows a stroke or head trauma and may be due to damage to a speech center in the brain that changes the speed, pitch, stress and pronunciation of words. The perception that the strange speech pattern is a foreign accent lies "in the ear of the beholder," according to researchers in Belgium. When they asked 33 students to place the accent of one FAS patient, they got 10 different answers, ranging from Turkish to Romanian.

Intermittent Explosive Disorder. As many as 1 in 14 people (mostly men) have angry, aggressive or violent outbursts, way out of proportion to situations, as in road rage and domestic abuse. Episodes generally last from 10 to 20 minutes, may be accompanied by chest tightness or heart palpitations and are often followed by embarrassment or remorse. Experts think IED may involve an imbalance of serotonin and testosterone, and it tends to run in families, though it may be as much environmental as genetic: People raised in violent homes seem particularly prone to it.

Alien Hand Syndrome. Fans of "Dr. Strangelove" will recall the title character's inability to control his right hand, which kept trying to give a Nazi salute. Real-life sufferers of AHS (only a few dozen to date) lose conscious control of a limb, probably due to a lost connection between brain hemispheres. The "alien" hand may thwart what the other hand is doing, such as unbuttoning a shirt the other hand is buttoning, or tamping out a cigarette the other hand has just lit. Symptoms can be managed by keeping the rogue hand preoccupied by giving it an object to hold or by muffling it with an oven mitt.

Capgras Delusion. The conviction that a close friend or family member is an impostor was named for the French psychiatrist who first described it in 1923. Experts now think it stems from a disconnect between the regions of the brain that perceive faces and register emotional responses. About one-third of people with dementia have Capgras at some point. A related disorder, Cotard's Delusion, causes sufferers to think they are dead, decaying or never existed at all. Both disorders have formed the plot of novels, TV shows and movies, from an episode of "Scrubs" to the new film "Synecdoche." Antidepressants and electroconvulsive therapy sometimes help.

Spasmodic Dysphonia. The inability to speak except in rhymes, whispers or a falsetto voice may seem like a gag worthy of "Dilbert." But the comic strip's creator, Scott Adams, is among some 30,000 Americans who have SD, in which spasms prevent the vocal cords from vibrating normally. Botox injections around the larynx can calm the spasms temporarily. The condition seems to vanish when sufferers sing, recite poetry or change the tenor of their voice. Mr. Adams read nursery rhymes aloud every night in an effort to "remap" his brain and was able to recover much of his speaking ability.

Alice in Wonderland Syndrome. Named after Lewis Carroll's famous novel, this neurological condition makes objects (including one's own body parts) seem smaller, larger, closer or more distant than they really are. It's more common in childhood, often at the onset of sleep, and may disappear by adulthood. The prevalence and origin are unknown, but it sometimes accompanies migraine headaches, epilepsy, brain tumors or the use of psychotropic drugs.

Synesthesia. In another form of scrambled perception, some people have an odd mixing of the senses in which they strongly associate certain numbers with colors, sounds with tastes or other sensory variations. As many as 1 in 200 people may have some form of it, often without realizing it. Scientists think the condition results from cross-wiring in the brain, with neurons and synapses from one sensory system crossing over into another. One theory holds that most infants have such crossed connections but gradually lose the associations in adulthood. Synesthesia is generally harmless, and often associated with creativity.

Jumping Frenchmen of Maine Disorder. The typical response to being startled -- muscles tense, heart pounds, senses go on alert -- lasts only a few seconds. But in this disorder, first observed in 1878 among French-Canadian lumberjacks in the Moosehead Lake area of Maine, the reaction is greatly exaggerated. Sufferers jump, twitch, flail their limbs and obey commands given suddenly, even if it means hurting themselves or a loved one. It's also been observed in factory workers in Siberia and Malaysia. Some experts believe it's a genetic mutation that blocks glycine, a neurotransmitter that calms the central nervous system's response to stimuli. Others think it's more psychological than neurological, and perhaps part of a heightened defense mechanism from living and working in close quarters.

Stendhal Syndrome. The tendency to develop a rapid heartbeat, dizziness and hallucinations when exposed to great art seems like a great exit line for tired museum-goers. But it seems particularly prevalent in Florence, Italy. An Italian psychiatrist observed it in more than 100 visitors in the 1970s and named it after the French author, who described similar symptoms upon visiting Florence in 1817. More than 100 additional cases have been documented, including some in which a particular detail of a painting seemed to bring on acute anxiety. Effects are usually temporary.

Paris Syndrome. This variation of Stendhal Syndrome primarily affects Japanese tourists; about a dozen a year experience a psychiatric breakdown in the City of Light. In the 1980s, a Japanese scientist theorized that the disorientation is brought on by the combination of exhaustion, the language barrier and difficulty some Japanese have reconciling their idealized vision of Paris with the modern reality.

Jerusalem Syndrome. Reports of visitors to Jerusalem being seized by a religious psychosis date back to the Middle Ages. In the 1930s, Israeli psychiatrists formally described the condition in which tourists assume the persona of a Biblical character and make strange pronouncements at a holy site. (In one 1969 case, an Australian tourist set fire to a local mosque, sparking a citywide riot and a 1998 film of the same name.) An article in the British Journal of Psychiatry in 2000 noted that 1,200 patients had been referred to a central mental facility with the disorder between 1980 and 1993. Some had previous mental disorders; others seemed quite sane before and later resumed normal lives. The authors warned tour guides to be alert for the disorder's seven characteristic stages: Sufferers appear agitated; seek to leave the group to tour Jerusalem alone; become obsessed with purity and cleanliness; prepare a toga-like gown "often with the aid of hotel bed linen"; shout Bible verses, then march to a holy site and deliver a confused sermon that often appeals to humankind to adopt a more moral way of life.


No comments: