Vote Now for the best name in Minor League Baseball

Remember to watch these 5 players at the MLB Futures Game

By David Brown

The presentation of the Futures Game has never quite matched the inspired concept.

Getting the best almost-ready prospects from across the league into one game, dividing the players into a "U.S. against the World" competition, was a great idea by league exec Jimmie Lee Solomon.

A year ago, if you had attended at Busch Stadium in St. Louis or tuned your TV or satellite radio properly, you could have watched the likes of Jason Heyward, Mike Stanton, Carlos Santana, Neftali Feliz, Pedro Alvarez and Mat Latos.

Before they were stars!

IF you had tuned in, that is. But you probably didn't tune in because MLB starts the game on a Sunday afternoon before the All-Star break when, as we all know, the first half of the season is going on. And the stupid celebrity softball game seems to get as much pub.

Regardless, if I'm a Braves fan in 2009, I'm going to watch — you know — the BRAVES on Sunday afternoon, even at the expense of teen Jason Heyward.

This season, if I am a Royals fan (Do they really exist?), I am going to watch the R-R-R ... OK, maybe I tune in for Mike Moustakas, who was among the 25 players named to the U.S roster today. But they're the exception!

Until the schedulers air the Futures Game in prime time, with no actual major league games competing for attention, it will remain one of the best-kept secrets in baseball.

In order to whet your appetite for the future, here are the five top of the top prospects for whom to watch (if you happen to watch):

U.S. ROSTER
• Moustakas 3B: (.355/.421/.704 at Class AA) Just this guy's last name has "future star" written all over it. Standing 5-foot-11, weighing 230 pounds, KC's third baseman of soon would give the Royals one of the best young corner combos (with Billy Butler) in the majors. He's batting .422 with runners in scoring position. Say it with me: "Mooooooose!"

• Domonic Brown OF: (.317/.389/.585 at Class AA) Lanky, speedy and exceedingly polite, he's probably ready to replace Raul Ibañez — or Jayson Werth — if the Phillies need him.

• Jeremy Hellickson RHP: (2.33 ERA, 90 K, .234 BA against at Class AAA) The Rays' ace of tomorrow averages a strikeout per inning with great control. The Yankees and Red Sox want no part of him.

WORLD ROSTER
• Julio Teheran RHP: (1.26 ERA, 94K in 78 1/3 IP at Class A) A native of Colombia with an Iranian-sounding last name, he is having a breakthrough season for the Braves at age 19. Second-youngest player on either roster, after Los Angels Angels outfielder Mike Trout.

• Carlos Peguero OF (.301/.388/.531 in Class AA) The Mariners could use this guy in the lineup right now. I'm getting a Ruben Sierra feel from Peguero, a 6-foot-5 left-handed batter who is from Hondo Valle (Hondo? Are you KIDDING?) in the Dominican. He's got 28 extra-base hits in 256 at-bats after finishing with 66 XBHs (including 14 triples) a year ago. He's lowering his strikeout rate (had 172 last year) but he's struggling against LHP. Facially, he looks like Albert Pujols. Just turned 23.

Under the Microscope: How Fast Can a Human Run?

Speed limits

Researchers think 30mph could be the human limit. Most use the 100m to calculate how fast we can run. The current record for the 100m is 9.58 seconds, by Usain Bolt in 2009. That gives a speed of 23.3mph. But interestingly, during the 60-80m stretch of the race Bolt averaged a speed of 27.8mph.

However, as Neils Bohr, the distinguished physicist, said: "Prediction is very difficult, especially if it is about the future". Many commentators previously suggested under 10s was unbreakable; now Bolt's coach is aiming for 9.4s. The 100m record now held by a woman would have beaten the fastest male back in the Fifties.

How to run fast

A key thing is the use of the chemical energy ATP (adenosine triphosphate) in our muscles. This fuels them to contract, to move the various joints in our legs, to forcefully hit the ground and turn that energy into speed. For sprinting, having fast-twitch muscle fibres, which quickly translate energy into force, gives you an advantage.

Recent Polls

Recent Polls:

Poll Ending on 5/14/2010:
Question: Do you like the new look?
Answer: Yes - 100%

PollEnding on 5/30/2010:
Question: What would you like to see more on Chickens on the Run?
Answer: _____

Slugger Museum Offers Glimpse Into Baseball's Past

LOUISVILLE, Ky. — Grabbing a bat once swung by Johnny Bench brought a rush of cherished memories for James Montgomery during a visit to the Louisville Slugger Museum & Factory — where baseball memorabilia mixes with a close-up look at how the famed bats are crafted.

Just touching the bat used by the Hall of Famer had Montgomery reliving excursions to the ballpark with his father decades ago to watch his hometown Pittsburgh Pirates try to outslug the Cincinnati Reds and their "Big Red Machine" lineup that included Bench.

"That was pretty neat holding that bat," Montgomery said after touring the museum and factory with his wife and in-laws. "It brings back those childhood memories."

Hillerich & Bradsby Co. — a fifth-generation, family-owned company — has been crafting bats since 1884 and currently churns out about 1.8 million Louisville Sluggers each year.

About six in 10 big-leaguers currently use Louisville Slugger bats.

The sights and smells can tantalize visitors to the museum and factory — a must-see for baseball fans if they happen to be anywhere near Kentucky's largest city. Towering outside is a 120-foot-tall steel bat that looms as a landmark in downtown Louisville.

Inside, visitors stroll through the heart of the factory — where fast-churning machines turn cylinder-shaped billets of wood into bats used from recreational leagues to the big leagues. The factory is enveloped by the smell of the wood and the whirring sound of the equipment.

"You're really in the factory," said Montgomery, of suburban Dallas, whose family was in town for a swimming competition. "That was really cool. A lot of times you're like on a sky bridge and they're all the way down on a lower level.

"You're right there — you hear it, you smell it, you see it."

Next to the factory is the museum, which features a treasure trove of bats once gripped by a "Who's Who" of baseball greats. There are lots of photos and interactive displays.

The most eye-catching display might be a bat used by Babe Ruth in 1927, when he hit 60 home runs. The 40-ounce bat has 21 notches carved along the top of the Louisville Slugger logo — one for each round-tripper that Ruth slugged with the bat.

Another display shows the Louisville Slugger that Hank Aaron used for his 700th home run on his way to surpassing Ruth on the career home run list. Opening a drawer in the same case reveals bats used by such greats as Cal Ripken Jr., Eddie Murray, Mike Schmidt and Tony Gwynn.

Just a short toss away is a bat used by Ted Williams late in his fabled career, and a nicked bat once swung by the tragic "Shoeless" Joe Jackson, who was banished from baseball along with several teammates after being accused of conspiring to throw the 1919 World Series.

2K Sports Hurls $1M "Perfect Game" Challenge

Mar 4, 2010 6:04 AM, Brian Quinton for PROMO Xtra

As a promotion for the latest version of its new baseball videogame, 2K Sports has chosen quite a pitch: $1 million to the first registered player who offers documented proof that he or she pitched a perfect game on MLB 2K10, this year’s update of the videogame for the Xbox 360 and PlayStation 3 consoles.

The promotion is notable not only for the size of the offer—reportedly one of the largest cash awards ever—but because it involves a skill challenge rather than simple entry into a random drawing.

And skill is at the heart of the campaign. Game maker 2K Sports, a division of Take-Two Interactive, has revamped this year’s version to add more responsive controls for pitching, and the promotion is designed to highlight those features. Players can now choose a pitch type and then execute that pitch with joystick control, permitting more realistic variance in pitching and in effect turning MLB 2K10 into a pitchers’ duel.

Players must pitch that perfect game between March 2 and May 1 2010 and must document their entire game on video. To enter, they must purchase an authorized copy of the game for Xbox or PlayStation and click on the MLB 2K10 Contest prompt at startup.

For purposes of the contest, 2K Sports defines a “perfect game” as one without any pitcher substitutions in the game play, no pauses during play, no delays of more than 60 seconds between pitches, and no mound visits from the coach. All 27 batters in nine innings must be retired without letting any one reach base.

The manufacturers have also imposed explicit instructions about videotape proof of completion, including requiring that the quality is good enough to read the game controls on the monitor screen and turning the camera on at least 5 seconds before starting up the TV and game console. Those instructions are available at www.2ksports.com/perfectgame, including FAQs and a downloadable .pdf checklist. Players can also record their game digitally.

A player who completes a perfect game in this mode will instantly receive a verification code which must be input at the perfect Game Web site. They will then have to follow up by furnishing the entire recording in DVD form to the judges at 2K Sports to be eligible for the prize.

2K Sports marketing director Chris Snyder told the Kotaku gaming blog that the company is serious about handing out $1 million for the first perfect game in the contest period, and in fact expects to have to do so.

“I would be shocked if someone doesn’t get it,” he said. “It’s very doable. It’s been budgeted for. It’s in an escrow account, and we’re fully expecting to pay it.”

Still there’s no denying it will be tough, if real-world experience is any indication. Only 18 perfect games have been pitched in recorded major league baseball history, and two of those happened before 1900.

Confidence Builder

If you ever feel a little bit stupid, just dig this up and read it again; your confidence will be restored!

'Outside of the killings, Washington has one of the lowest crime rates in the country,'--Mayor Marion Barry, Washington, DC

'Whenever I watch TV and see those poor starving kids all over the world, I can't help but cry. I mean I'd love to be skinny like that, but not with all those flies and death and stuff.'--Mariah Carey

'We've gotto pause and ask ourselves: How much clean air do we need?' --Lee Iacocca

'The word 'genius' isn't applicable in football. A genius is a guy like Norman Einstein.'--Joe Theisman, NFL football quarterback & sports analyst.

(On September17, 1994, Alabama's Heather Whitestone was selected as Miss America 1995.)

Question: If you could live forever, would you and why?

Answer: 'I would not live forever, because we should not live forever, because if we were supposed to live forever, then we would live forever, but we cannot live forever, which is why I would not live forever,'--Miss Alabama in the 1994 Miss USA contest.

'We don't necessarily discriminate. We simply exclude certain types of people.'--Colonel Gerald Wellman, ROTC Instructor.

'I've never had major knee surgery on any other part of my body,'--Winston Bennett, University of Kentucky basketball forward.

'That lowdown scoundrel deserves to be kicked to death by a jackass, and I'm just the one to do it,'--A congressional candidate in Texas

'Half this game is ninety percent mental.'-- Philadelphia Phillies manager, Danny Ozark

'Your food stamps will be stopped effective March 1992 because we received notice that you passed away. May God bless you. You may reapply if there is a change in your circumstances.'--Department of Social Services, Greenville , South Carolina

'I love California. I practically grew up in Phoenix .'--Dan Quayle

'Traditionally, most of Australia 's imports come from overseas..'--Keppel Enderbery

'Smoking kills. If you're killed, you've lost a very important part of your life,'--Brooke Shields, during an interview to become spokesperson for federal anti-smoking campaign .

'If somebody has a bad heart, they can plug this jack in at night as they go to bed, and it will monitor their heart throughout the night. And the next morning, when they wake up dead, there will be a record.'--Mark S. Fowler, FCC Chairman

Why Children Shouldn't Have Camera's

This is just HILARIOUS to me. What I am going to do is update the post, repost it everyday with the latest, adding it underneath the previous day's strip to follow the progress. Why? Just because.

Home Plate

Game 54: Mavs at Rockets

I wanted to give an update to some teams listed on this site:

Texas Tornados: 1 Year off for facility rebuilding
Dallas Desperados: League stopped. Future unknown.
Austin Ice Bats: 1 year off for facility rebuilding
Dallas Diamonds: Season starts April 11th
Texas Wildcatters: I don’t know what in the world is going on with them. Disbanded?

Houston Comets: Disbanded from league.

The Mavericks are coming into this game winners of three of their last four games, with the last game coming against the Nets in which they won 113-98. Jason Terry is out with an injury dating back to February 7th and will be out six weeks from that date, which is March 21st. Houston has a 1/2 game lead over the Mavericks in their division. This game has a direct impact on the standings.

Mavericks Stats 08-09

Name	Dirk Nowitzki	Josh Howard	Jason Kidd	Brandon Bass
Points Per Game	25.9	17.9	9.1	8.3
Rebounds Per Game	8.3	5.1	6.3	4.4
Assists Per Game	2.3	1.8	8.5	0.5
Blocks Per Game	0.8	0.6	0.6	0.7

It looks weird without Terry on this list. Still, having Brandon Bass listed on here does show that this team has some strength beyond the top four. Still, missing a guy who brings 19.9 points a game hurts.

Rockets Stats 08-09

Name	Yao Ming	Ron Artest	Luis Scola	Rafer Alston
Points Per Game	19.8	15.9	12.3	11.5
Rebounds Per Game	9.6	5.2	8.1	3.0
Assists Per Game	1.7	3.1	1.3	5.4
Blocks Per Game	1.7	0.3	0.1	0.2

The Rockets are missing Tracy McGrady. In comparison to the Mavericks Terry, Terry averages more points a game than Yao Ming. Still, McGrady puts up 15.6 points a game and averages 5 assists a game. I'm not really sure who I would prefer to have on my team, comparing the two. I know I would want Yao Ming over any center the Mavericks have right now. But that is beside the point.

Forecast: These two teams are 1-1 against each other this season. Both teams have won three of their last four, with the Mavericks loss coming against Boston and Houston's loss coming against Milwaukee. Boston has the #2 record in the NBA. Milwaukee is three games under 500 and seeded 8th in the Eastern Conference right now. The edge goes to Mavericks there. For whatever reason, my gut is saying the home team on this one. Houston has the fourth best home record in the West and seventh in the NBA. Not bad. Rockets take this one, 99-96.

Home Court

Pictures of a Baseball

What is Important to Me

Well, a friend of mine asked for me to take a picture of something important to me. There was one restriction, which was it could not be a picture of family. So, what is important to me besides a relationship with God and my family? Well, friends and baseball. I figured friends would fall into the family catagory, so I took pictures of a baseball and then made a collage out of it. Here are two pictures that I made up for my friend:I think I am going to use the picture with the six pictures of the baseball.
I have many, many more pictures of this baseball... limiting it to just six or seven of the pictures I took made it a really difficult task to choose which pictures to use and which ones to not use. All in all, I took 21 pictures of a baseball in different locations and different angles. It was a fun little project. I will post more pictures of the baseball soon. Until then, enjoy.

Home Plate

Merry Christmas

I just want to wish everyone a Merry Christmas, keep it safe and have fun. I am just hopeful that the two can mix.

Home Plate

New for 2009

This is the new header for 2009. I just went ahead and put it up now... as far as I know, these are all the Professional Logo's in the DFW area, with the exception of the SGP Warriors and Haltom Buffaloes logos, both of which are high schools...Here are the answers to the the "Logo Madness" posted at the start of this month.

Last, but not least, is my Wallpaper on my computer. It has most of the ticket stubs from 2008. I think it is very cool, personally, and thought I shouldn't keep it all to myself. If you want, save it, you know. If you don't... well, don't :-]

Home Plate

Johnny's Mad Lib

The __________(Sports Team) are prepared for __________(Name, First and Last, #1) to sign elsewhere as a free agent, but if he goes the club will at least receive a memento of his time in ________(Place).

By offering ______(Last Name, #1), a Type ___(Letter of Alphabet) free agent, salary arbitration Monday, the __________(Same Sports Team) ensured they will receive a draft pick in ________(Noun, with a or an) if the AL's on-base-plus-slugging percentage leader signs with another club.

The __________(Same Sports Team) did not offer _____(Noun, Plural) to their other free agents: infielder __________(Name, First and Last, #2) and right-handers __________(Name, First and Last, #3) and ___________(Name, First and Last, #4). None of them were ranked free agents, so the ___________(Same Sports Team) would not have ______(Verb, past tense) compensation for them.

The club can continue to ________(Verb) with all four, if it so chooses. The Rangers do have interest in bringing ________(Last Name, #1) back on a ___(Number) or ___(Higher Number) year deal, but ______(Title) __________(Name, First and Last, #5) has said for weeks he expected _____(Last Name, #1) to fully explore the ________(Event). The club does have interest in bringing _____(Last Name, #3) back on a minor league contract if he proves he is finally ______(Adjective).

Home Plate

Logo Madness: Part I

I figured I would try some trivia for my readers. Above are 10 logos... can you name them all? I figured I would start with something easy and then next month, we would have something much, much harder.

The first to post with the correct answer will win a no-prize. Good luck to you all!

Home Court

Online Predictions

My online poll consisted of twelve votes:

• Less than 71 wins: 2 Votes
• Between 71-76 wins: 0 Votes
• Between 77-81 wins: 1 Vote
• Between 82-87 wins: 6 Votes
• Between 88-95 wins: 1 Vote
• Over 95 wins: 2 Votes

I think it is definitely safe to say that "Mr. Anonymous" voted in the "less than 71 wins"... still, the group 82-87 wins... that is refreshing to see. I don't see the Rangers finishing with less than 71 wins, personally... and that is me trying to not be biased in my opinion. The loss of Ponson is hard, but the Rangers will recover from that. It didn't look like he would've been too much of an asset at this point anyway.

This is a logo the Rangers haven't been using, it is an alternate logo that hasn't seen the light of day...

Home Plate

A Letter from Brother to Sister

Hi Sue,
Just another note from your bottom-dwelling brother. Last week I had a bad day at the office. I know you've been feeling down lately at work, so I thought I would share my dilemma with you to make you realize it's not so bad after all. Before I can tell you what happened to me, I first must bore you with a few technicalities of my job. As you know, my office lies at the bottom of the sea. I wear a suit to the office. It's a wetsuit. This time of year the water is quite cool, so what we do to keep warm is this: We have a diesel powered industrial water heater. This $20,000 piece of equipment sucks the water out of the sea. It heats it to a delightful temperature. It then pumps it down to the diver through a garden hose, which is taped to the air hose. Now this sounds like a darn good plan, and I've used it several times with no complaints. What I do, when I get to the bottom and start working, is take the hose and stuff it down the back of my wetsuit. This floods my whole suit with warm water. It's like working in a Jacuzzi.

Everything was going well until all of a sudden, my butt started to itch. So, of course, I scratched it. This only made things worse. Within a few seconds my butt started to burn so I pulled the hose out from my back, but the damage was done. In agony I realized what had happened. The hot water machine had sucked up a jellyfish and pumped it into my suit. Now, since I don't have any hair on my back, the jellyfish couldn't stick to it. However, the crack of my butt was not as fortunate. When I scratched what I thought was an itch, I was actually grinding the jellyfish into the crack of my butt. I informed the dive supervisor of my dilemma over the communicator. His instructions were unclear due to the fact that he, along with five other divers, were all laughing hysterically.

Needless to say I aborted the dive. I was instructed to make three agonizing in-water decompression stops totaling thirty-five minutes before I could reach the surface to begin my chamber dry decompression.

When I arrived at the surface, I was wearing nothing but my brass helmet.

As I climbed out of the water, the medic, with tears of laughter running down his face, handed me a tube of cream and told me to rub it on my butt as soon as I got in the chamber. The cream put the fire out, but I couldn't poop for two days because my butt was swollen shut.

So, next time you're having a bad day at work, think about how much worse it would be if you had a jellyfish shoved up your butt.

Totally not related to baseball (or sports, for that matter), but very funny and it puts a bad day into perspective. Johnny Out.

A Chicken Tale

This story, well, I just couldn’t leave it off of this blog. I mean, this is the “Chickens on the Run,” and while I have my reasons for the name of this blog, strange as they may be, when I was out for a walk Friday evening or Saturday morning, however you may want to look at, this story is simply fitting. And hilarious as well, we can’t leave that out. OK, so, I finished my quiet time (which is another blog I have) and normally every evening or at least several times a week, I journey for a walk around the apartment complex that my family lives in. Most importantly in apartment complex, in back part, you will find a variety of wild creatures, such as raccoons, possums, and rarely armadillos. Well, on this fine evening, I ventured out at about 1 AM, which is very, very late for me. I generally take my walks somewhere between 10 to 11 PM. So I go out walking and there are these cop cars driving all over place and policemen running all around. There aren’t any gunshots and no one is yelling… so I keep walking. But I do wonder “should I go back inside?” I keep walking. Everyone is gone… it’s just me. No creatures of the night, raccoons are asleep I guess. Mr. Possum is playing dead. Then some kids come running past. They turn and disappear. And then some cops drive by. And then a policeman runs past. And then another policeman runs up to me and asks, “Have you seen some kids run by?” And I point the policeman in the kids’ direction.” And I keep walking. And as I walked, I came across something rather strange, something you really just don’t see in a city such as Dallas, a big pillow like ball of feathers on a windshield of a car. I didn’t know what to make of it. And noting the immediate events that had just occurred I was rather leery. So I crept up a little and circled the car from a safe distance. It didn’t look to have a head or tail. It was a ball of feathers, literally. So I crept up a few feat more, still circling, and I had then determined it was or used to be a chicken, because it has wings, but still no head (what sick person would decapitate a chicken and then throw it on someone’s car?). I crept up a few feet more, now only but a foot or two from the car, and low and behold it does have a head, but it doesn’t look alive. I guess this person hit the chicken while driving home, maybe drunk or something, and just left it there and crashed and went to bed? It could happen. And then, someone turned the corner and their headlights illuminated the scene just enough to let know that this poor chicken was alive and well. And I laughed. And I thought to myself, “I’m going to take this chicken home to my apartment and then give to my parents in the morning, because they will know what to do it with it.” And as I walked to my apartment, I continued to laugh… because I was bringing a chicken home. I placed the bird on my patio. And then washed my hands and went to bed. I nudged my wife and let her know the bird was on the patio, and she just muttered to me, “I just hope it’s not a rooster.”

And then a little after 6 AM we found out it in fact was a rooster… and my first thought was, “OH MY GOD, I’M GOING TO GET EVICTED!!!” I quickly started getting dressed and I called my parents to see if I could bring the bird over to which my father replied, “We don’t need a chicken.” But they took the bird anyway. My father continued to remind me of the time of the call “6:17 A.M.” which I am sure I will continue to hear about for a good while. I am surprised that there wasn’t an angry mob outside my door when I carried the bird to car, but I was greeted by a few neighborhood cats, looking for the bird. In fact, when I went to but the rooster in the cat carrier, there was one climbing the fence, which I had to push back over. A very interesting spread of few hours. Interesting indeed.

Also, for you Mavs fans out there, the Mavs will win game 7 tonight, why? Because my son is wearing Mavs gear and when he wears Mavs gear, they win. They are undefeated when he wears a particular sports clothing, which is weird. He wears Rangers clothing, the Rangers win, he wears Mavs clothing, the Mavs win. I had to choose between Kameron Loe tonight or the Mavs and Game 7 and I am sure the Loe-Rider will forgive me on this one. So, alas, Johnny Out.

Lou Gehrig's disease

Lou Gehrig’s disease: I thought I would cover this, since three of my four Bush League teams are in his league.

Overview

Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, is a progressive neuromuscular disease that weakens and eventually destroys motor neurons (components of the nervous system that connect the brain with the skeletal muscles). Skeletal muscles are involved with voluntary movements, such as walking and talking. The motor neurons transmit the command to move from the brain to the skeletal muscles, which respond by contracting.

A person with ALS usually presents with problems in dexterity or gait resulting from muscle weakness, or with difficulty speaking or swallowing. Sphincter control, sensory function, intellectual ability, and skin integrity are preserved. Patients become paralyzed and often require ventilation and surgery to provide a new opening in the stomach (gastrostomy). Loss of respiratory function is ultimately the cause of death.

Incidence and Prevalence

Approximately 30,000 patients in the United States currently have ALS. The disease has no racial, socioeconomic, or ethnic boundaries. The life expectancy of ALS patients is usually 3 to 5 years after diagnosis. ALS is most commonly diagnosed in middle age and affects men more often than women.

Risk Factors

Risk factors include an inherited genetic defect, which accounts for 5–10% of cases of familial ALS (FALS) in the United States. FALS is linked to a genetic defect on chromosome 21. This gene codes for an enzyme called superoxide dismutase (SOD), an antioxidant that protects motor neurons from free radical damage (i.e., molecules introduced to the body, or produced by body processes that interact and cause cellular damage). More than 60 different mutations that cause SOD to lose its antioxidant properties have been found. However, only 40% of familial ALS cases are linked to SOD mutations, so there may be other unknown genetic defects involved.

In the United States, 90–95% of ALS cases are sporadic. Sporadic ALS appears to be increasing worldwide. The causes are not clear, yet some evidence suggests that the immune system may be involved. Excessive levels of glutamate can overstimulate motor neurons and cause them to die. Glutamate is one of the most important neurotransmitters for healthy brain function. Neurotransmitters are chemicals that transmit signals from one nerve to another.

In Guamanian ALS, a dietary neurotoxin is the risk factor. The suspected neurotoxin is an amino acid (BMAA) found in the seed of the cycad Cyas cirinalis, a tropical plant found in Guam, which was used to make flour and was a major dietary component during the 1950s and the early 1960s, when this type of ALS had an exceptionally high incidence.

Causes

The cause of ALS is not completely understood. Researchers and physicians suspect viruses, neurotoxins (especially in Guamanian ALS), heavy metals, DNA defects (especially in familial ALS), immune system abnormalities, and enzyme abnormalities.

Signs and Symptoms

Once a motor neuron degenerates completely, the muscle that it controls no longer receives impulses from the brain. Approximately 60% of ALS patients experience muscle weakness and stiffness as the initial symptom. Usually the first muscles affected are those in the arms and legs. Walking or climbing stairs may be difficult. The patient may drop things, fall, experience muscle cramps, and laugh or cry uncontrollably. The arms and legs may feel especially tired. If the hands are affected, the patient may have difficulty picking up small objects or turning keys. Speech problems, such as slurring, hoarseness, or decreased volume may also occur.

Signs and symptoms include the following:

• Absence of spinal reflexes (areflexia)


• Loss of muscle tone (hypotonia)


• Muscle twitching (fasciculations)


• Muscle wasting (atrophy)

The signs that the upper motor neurons are affected include the following:

• Excessive salivation


• Extension of the big toe and abduction of the rest of the toes in response to lightly stroking the sole of the foot (Babinski's sign)


• Reduced muscle tone, or hypotonia, and rigidity (spasticity)


• Hyperactive tendon reflexes


• Impaired speech (dysarthria)


• Impaired swallowing (dysphagia)


• Rapidly alternating muscle contractions and relaxations (clonus)

As symptoms progressively worsen, the patient's muscles atrophy causing spasticity, stiffness, abnormal movements, and alterations in gait and manual dexterity. Twitching may occur in the tongue and in affected limbs. The patient may experience muscle pain and muscle cramps. Some patients experience more difficulty swallowing saliva and liquids than solid food. Excessive salivation and difficulty swallowing may cause drooling. When respiratory muscles weaken, the patient may require a ventilator.

ALS patients often experience fear, anxiety, and depression.

Diagnosis

ALS is difficult to diagnose because the symptoms are similar to those of other neuromuscular disorders, many of which are treatable. The diagnosis is usually based on a complete neurological examination and clinical tests.

The neurological exam usually shows evidence of muscle weakness (localized or widespread, depending on the extent of the disease). The exam also reveals muscle atrophy. The muscles may be so stiff that when the neurologist moves them, they continue to move abnormally afterward. When the neurologist tests the "knee jerk" reaction, the movement is abnormally quick.

Because ALS affects the skeletal, voluntary muscles, the neurological exam does not reveal abnormalities in the sensory reflexes (i.e., vision, hearing, taste, smell, touch, or bowel and bladder control).

Tests

Nerve conduction velocity (NCV) and electromyography (EMG) help diagnose nerve and muscle disorders. These tests, which can be used in combination, are often referred to as EMG/NCV studies. NCV is administered before EMG and measures the speed at which nerves transmit electrical signals.

During NCV, electrodes are placed on the skin over a nerve that supplies a specific muscle or muscle group. A mild, brief electrical stimulus is delivered through the electrode and the response of the muscle is detected, amplified, and displayed. The strength of the signal is also measured. Neurological conditions can cause the NCV to slow down or to be slower on one side of the body. The strength of the response also provides clues to help with diagnosis and to determine the extent of the disease.

EMG measures nerve impulses within the muscles. Tiny electrodes are placed in the muscles in the arms and legs and the electronic responses are observed using an instrument that displays movement of an electric current (oscilloscope). As muscles contract, they emit a weak electrical signal that can be detected, amplified, and tracked, providing information about how well the muscles are working. These responses are abnormal in cases of ALS.

Tests may be performed to rule out other neurological disorders. Magnetic resonance imaging (MRI scan) may be used to rule out spinal cord disease. Blood tests may be done to detect the presence of heavy metals such as lead in the blood. Laboratory tests may detect abnormal proteins or hormone levels associated with other neurological diseases. A lumbar puncture or spinal tap may be performed to analyze the cerebrospinal fluid for genetic abnormalities (e.g., viral, autoimmune, neurotoxic).

Treatment

There is no cure for ALS. Treatment focuses on relieving symptoms and maintaining an optimal quality of life. Treatment is based on individual therapy and the continual adaptation of medications. Riluzole (Rilutek®) is one of the few drugs effective against ALS and may prevent progression and prolong life for a few months or so. Riluzole decreases the release of glutamate. Side effects include the following:

• Dizziness


• Elevated liver enzymes


• Reduced leukocytes in the blood (granulocytopenia)


• Weakness (asthenia)

Baclofen (Lioresol®) or tizanadine (Zanaflex®) may relieve spasticity. Side effects include increased weakness, sedation, and dizziness. Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen may relieve general discomfort. Due to potentially severe gastrointestinal and cardiovascular side effects, NSAIDs should only be used as instructed. Tramadol (Ultram®) is often prescribed for pain relief.

Physical therapy is an important part of treatment and helps to relieve cramping and muscular pain. Passive stretching helps to avoid permanent contraction of muscles (contractures) that may cause joint problems.

Other therapies, such as occupational therapy and speech therapy, are also used in treatment. ALS patients require a diet of high-energy foods that are easy to swallow. Patients may benefit from a nutritionist. If the patient is not able to maintain adequate nutrition, a percutaneous endoscopic gastrostomy (PEG), or feeding tube, is usually inserted.

Some ALS patients may also need pulmonary consultants and respiratory therapists to assist breathing. Fewer than 5% of patients use long-term ventilation support.

Depression is very common among ALS patients. Antidepressant medication and counseling can help patients and their families cope.

Prognosis

ALS is a terminal illness. Fifty percent of patients die within 3 years of diagnosis; 20% live 5 years; and 10% live 10 years. Hospice care can provide comfort and dignity to patients and their loved ones.

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