C Winterholler Popular Books
C Winterholler Biography & Facts
Hereditary spastic paraplegia (HSP) is a group of inherited diseases whose main feature is a progressive gait disorder. The disease presents with progressive stiffness (spasticity) and contraction in the lower limbs. HSP is also known as hereditary spastic paraparesis, familial spastic paraplegia, French settlement disease, Strumpell disease, or Strumpell-Lorrain disease. The symptoms are a result of dysfunction of long axons in the spinal cord. The affected cells are the primary motor neurons; therefore, the disease is an upper motor neuron disease. HSP is not a form of cerebral palsy even though it physically may appear and behave much the same as spastic diplegia. The origin of HSP is different from cerebral palsy. Despite this, some of the same anti-spasticity medications used in spastic cerebral palsy are sometimes used to treat HSP symptoms. HSP is caused by defects in transport of proteins, structural proteins, cell-maintaining proteins, lipids, and other substances through the cell. Long nerve fibers (axons) are affected because long distances make nerve cells particularly sensitive to defects in these mentioned mechanisms. The disease was first described in 1880 by the German neurologist Adolph Strümpell. It was described more extensively in 1888 by Maurice Lorrain, a French physician. Due to their contribution in describing the disease, it is still called Strümpell-Lorrain disease in French-speaking countries. The term hereditary spastic paraplegia was coined by Anita Harding in 1983. Signs and symptoms Symptoms depend on the type of HSP inherited. The main feature of the disease is progressive spasticity in the lower limbs due to pyramidal tract dysfunction. This also results in brisk reflexes, extensor plantar reflexes, muscle weakness, and variable bladder disturbances. Furthermore, among the core symptoms of HSP are also included abnormal gait and difficulty in walking, decreased vibratory sense at the ankles, and paresthesia. Individuals with HSP can experience extreme fatigue associated with central nervous system and neuromuscular disorders, which can be disabling. Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. Symptoms of HSP may begin at any age, from infancy to older than 60 years. If symptoms begin during the teenage years or later, then spastic gait disturbance usually progresses over many years. Canes, walkers, and wheelchairs may eventually be required, although some people never require assistance devices. Disability has been described as progressing more rapidly in adult onset forms. More specifically, patients with the autosomal dominant pure form of HSP reveal normal facial and extraocular movement. Although jaw jerk may be brisk in older subjects, there is no speech disturbance or difficulty of swallowing. Upper extremity muscle tone and strength are normal. In the lower extremities, muscle tone is increased at the hamstrings, quadriceps and ankles. Weakness is most notable at the iliopsoas, tibialis anterior, and to a lesser extent, hamstring muscles. In the complex form of the disorder, additional symptoms are present. These include: peripheral neuropathy, amyotrophy, ataxia, intellectual disability, ichthyosis, epilepsy, optic neuropathy, dementia, deafness, or problems with speech, swallowing or breathing. Anita Harding classified the HSP in a pure and complicated form. Pure HSP presents with spasticity in the lower limbs, associated with neurogenic bladder disturbance as well as lack of vibration sensitivity (pallhypesthesia). On the other hand, HSP is classified as complex when lower limb spasticity is combined with any additional neurological symptom. This classification is subjective and patients with complex HSPs are sometimes diagnosed as having cerebellar ataxia with spasticity, intellectual disability (with spasticity), or leukodystrophy. Some of the genes listed below have been described in other diseases than HSP before. Therefore, some key genes overlap with other disease groups. Age of onset In the past, HSP has been classified as early onset beginning in early childhood or later onset in adulthood. The age of onsets has two points of maximum at age 2 and around age 40. New findings propose that an earlier onset leads to a longer disease duration without loss of ambulation or the need for the use of a wheelchair. This was also described earlier, that later onset forms evolve more rapidly. However, this is not always the case as De Novo Early Onset SPG4, a form of infantile HSP, involves loss of ambulation and other motor skills. Cause HSP is a group of genetic disorders. It follows general inheritance rules and can be inherited in an autosomal dominant, autosomal recessive or X-linked recessive manner. The mode of inheritance involved has a direct impact on the chances of inheriting the disorder. Over 70 genotypes had been described, and over 50 genetic loci have been linked to this condition. Ten genes have been identified with autosomal dominant inheritance. One of these, SPG4, accounts for ~50% of all genetically solved cases, or approximately 25% of all HSP cases. Twelve genes are known to be inherited in an autosomal recessive fashion. Collectively this latter group account for ~1/3 cases. Most altered genes have known function, but for some the function haven't been identified yet. All of them are listed in the gene list below, including their mode of inheritance. Some examples are spastin (SPG4) and paraplegin (SPG7) are both AAA ATPases. Genotypes The genes are designated SPG (Spastic gait gene). The gene locations are in the format: chromosome - arm (short or p: long or q) - band number. These designations are for the human genes only. The locations may (and probably will) vary in other organisms. Despite the number of genes known to be involved in this condition ~40% of cases have yet to have their cause identified. In the table below SPG? is used to indicate a gene that has been associated with HSP but has not yet received an official HSP gene designation. Pathophysiology The major feature of HSP is a length-dependent axonal degeneration. These include the crossed and uncrossed corticospinal tracts to the legs and fasciculus gracilis. The spinocerebellar tract is involved to a lesser extent. Neuronal cell bodies of degenerating axons are preserved and there is no evidence of primary demyelination. Loss of anterior horn cells of the spinal cord are observed in some cases. Dorsal root ganglia, posterior roots and peripheral nerves are not directly affected. HSP affects several pathways in motor neurons. Many genes were identified and linked to HSP. It remains a challenge to accurately define the key players in each of the affected pathways, mainly because many genes have multiple functions and are involved in more than one pathway . Axon pathfinding Pathfinding is important for axon growth to the right destination (e.g. another nerve cell or a muscle). S.... Discover the C Winterholler popular books. Find the top 100 most popular C Winterholler books.
Best Seller C Winterholler Books of 2024
-
Alpha
Sybil BartelBillionaire. Mercenary. Navy SEAL. The Teams trained me to be a killer. War taught me to be ruthless. Then an illfated mission proved I was human. Combat wounded, cut loose by the ...
-
Awaken Me
Jenna JacobHe was more than a ghost haunting my dreams…he was real. By day, I’m Julianna Garrett …a prim, proper accountant. By night, I’m tormented by dreams of an alpha, ambereyed Adonis wh...
-
The Seduction Series Boxset
Roxy Sloane“Sensual, thrilling and wild!”   Discover the bestselling series in one collection: THE SEDUCTION, THE BARGAIN, and THE INVITATION. Perfect for fans of Ana Huang, Sierra S...
-
Good Guy
Kate MeaderHe's a Special Forces veteran making his pro hockey debut. She's a dogged sports reporter determined to get a scoop. She's also his best friend's widow . . . Fa...
-
Inception of Gold
Lexy TimmsThings are not as bad as they seem. They are worse. Being the personal assistant to a narcissistic actress who just happens to own the key to my family's undoing isn't ex...
-
You Are Kind
Michael GordonA little kindness goes a long way. How can you help encourage your kids to be kind from a young age? Teach kindness to preschoolers Acts of kindness can be fun, easy, and make a ...
-
Escape, A New Life
David J AntocciTo save herself, she had to lose everything. Trapped in a tropical paradise with no memory of how she got there, Abby is thrust into a fight for her life. Hunted by a madman, and c...
-
Get Lucky
Lila MonroeFall for the hot and hilarious romcom spin on 'The Hangover', perfect for fans of Tessa Bailey, Ali Hazelwood, and Emily Henry! What happens when you wake up in a hotel s...
-
Salvation
Meghan O'FlynnIf you like mouthy detectives, serial killers, and suspenseful mysteries that don't quit, this chilling and actionpacked hardboiled detective series has you covered! Try this ...
-
Whiskey Girl
Melissa BelleThey'll do anything for each other…except commit. Logan Wild isn’t just the hottest broncriding cowboy in Texas. He’s also my best friendwith benefits. And that’s all we’ll ev...
-
Holy Bible
The Church of Jesus Christ of Latter-day SaintsThe 2013 edition of the Holy Bible contains all of the study aids contained in the 1979 edition and includes revisions to the study aids, several new photos, updated maps, and adju...
-
The Holy Bible - King James Version
King JamesHoly Bible King James Version Few Sample Paragraphs from The Holy Bible eBook, Genesis (OT) 1 Gen. 1 IN the beginning God created the heaven and the earth. 2 And the earth was with...
-
His Girl Next Door
Khardine GrayFrom USA Today Bestselling author Khardine Gray comes a sizzling hot single dad romance you won't want to miss. No strings attached fun with the ubergorgeous single d...
-
Think and Grow Rich
Greg Habstritt & Napoleon HillThink and Grow Rich is one of the most popular success book of alltime, having sold more than 60 million copies since it was first published more than 70...
-
The Odyssey
HomerAn Apple Books Classic edition. Homer’s eighthcentury epic poem is a companion to The Iliad . It tells the story of Odysseus, who journeys by ship for 10 years after the Trojan War...
-
Dream Psychology
Sigmund FreudAn Apple Books Classic edition. Written by the founding father of psychoanalysis, Sigmund Freud’s 1899 book is the definitive text on learning to interpret dreams. Freud’s groundbr...
-
The Stoic Mind
Addy Osmani & GoLimitlesssDiscover the timeless wisdom of Stoicism in a modern context with "The Stoic Mind," an enlightening visual guide by Addy Osmani and GoLimitlesss. This rich exploration co...
-
Noxious
Lexy TimmsStop setting yourself on fire to keep someone else warm. Brady and Levi have been together since high school, since before he became famous and started thinking only about himself....
-
Teased by Fire
Molly O'HareFrom USA Today bestselling author Molly O’Hare comes an enemiestolovers, older brother’s best friend, romantic comedy with a curvy, antisocial, supernaturalobsessed, allyearround h...
-
The Great Gatsby
F. Scott FitzgeraldAn Apple Books Classics edition. The Roaring Twenties are in full effect in F. Scott Fitzgerald’s riveting classic. Manabouttown Jay Gatsby seems to have it all, including loads of...
-
Always Yours
Claire RayeSome things are just meant to be... Ellen Somerville and Will McIntyre met by accident and under unusual circumstances. Getting sprayed by a skunk in a parking lot wouldn’t normall...
-
Become A Better Version of Yourself
Ben LeightonThis ebook contains golden nuggets on how to motivate, inspire and improve your current situation. It encompasses the holistic view of self improvement from mental& emotion...
-
Little Women
Louisa May AlcottAn Apple Books Classic edition. Meet the Marches! Louisa May Alcott’s classic introduces us to four unforgettable sisters: beautiful Meg, tomboyish Jo, delicate Beth, and Amy, the ...
-
Rogue Alpha
Kimber WhiteOne touch made her crave him. But the pull of fate could be the path to ruin. College student Laura Prince lands a plum internship deep in the Michigan wilderness. When she discove...
-
Becoming Lady Dalton
Carrie LomaxA dance of desire and deceit... In the glittering world of London's ton, Mrs. Viola Cartwright revels in her newfound freedom as a lady of leisureuntil a series of jewel theft...
-
The Three Little Pigs
Mark LeskyClassic fairy tales, legends and folk stories in short version without violence retold with lovely illustrations in simple language. Perfect for reading aloud to small chi...
-
Hard Love
Peyton BanksHe was football royalty. Legendary quarterback. A champion.  Reiner Strickland had led his team to three national championships and playing a knight in shining armor was a...
-
A Christmas Carol
Charles DickensAn Apple Books Classic edition. It’s Christmas Eve in Victorian England. While some families don’t know where their next meal will come from, Ebenezer Scrooge sits in his large hom...
-
A Bookshop to Die For
M.P. BlackDitching her fiancé at the altar, Alice Hartford bolts to her childhood hometown to reconnect with the last, happy remnant of her past: her mom's old bookstore. But the bookst...
-
Hot Off the Press
Lexy Timms"This is what really happened… reported by a free press, for a free people…" Wes Shaw leads a secret double life. As the secret owner of a billion dollar newspap...
-
All Fired Up
Kathryn ShayCaptain Jarek Zenko, a war veteran and firefighter, meets Lacey Roth at a bar one night. They don’t share their real identities, even when they retreat to a hotel. When they meet t...
-
Assisting the Bosshole
Kristin MacQueenNo hot water? Check Missed the train? Check Broke my heel? Check Dropped my coffee? Check My first day of my new job can’t possibly go worse, right? Wrong. When I meet Parker Scott...
-
The Scarlet Letter
Nathaniel HawthorneAn Apple Books Classic edition. Hester Prynne lives in infamy. After committing adultery and bearing a child with a man whose name she refuses to divulge, the heroine of Nathaniel ...
-
Saltwater Cove
Amelia AddlerSecond chances...and the secrets that sabotage them. At 48 years old, Margie Clifton never expected to be starting her life all over again. But when her brother gifts her a propert...
-
Law of Attraction
Jordan HollisHow to Finally Overcome the Hurdles of Manifesting Proven, effective and enjoyable ways to help you manifest faster… Right now, think of something that makes you successful. If you...
-
Pretty Little Lies
Ivy ThornFour years ago, Nicolo Marchetti took my innocence. But he left me with something else Nicolo almost took everything from me. My virginity, my reputation, and very nearly my future...
-
School of Potential
W.J. MayUSA Today Bestselling author, W.J. May brings you a continuation of the international bestselling series, The Chronicles of Kerrigan! Come back and enjoy the famous characters, or ...
-
Nothing to Hide
Scarlett FinnPrize of a lifetime: travel the world with a celebrity billionaire. Come to LA with us, Roxie… It will be so much fun! We have tickets for a latenight talk show! What could possibl...
-
The Four Loves
C. S. LewisThe Four Loves summarizes four kinds of human loveaffection, friendship, erotic love, and the love of God. Masterful without being magisterial, this book's wise, gentle, candi...
-
Masters of Restraint
Ines JohnsonMy new boss is good at giving orders. But his latest demand is NSFW. Especially when his two business partners want in on our deal. My new boss My boss’s boss Their investor &#...
-
Caught Up with the Captain
Kait NolanCan a retired naval commander and the love he left behind overcome a 34yearold secret to find their way to a second chance? Captain Mitchell Greyson is a man who believes in duty. ...
-
Pride and Prejudice
Jane AustenAn Apple Books Classic edition. Jane Austen’s beloved classic opens with this witty and very memorable line: “It is a truth universally acknowledged, that a single man in possessio...
-
Coffee Girl
Sophie SinclairMackenzie "Kiki" Forbes finds herself in a pickle. Either become her snarky sister's nanny, or move halfway across the country to work as assistanttothestylist of a ...
-
The Target
Lexy TimmsWhen you seek revenge be sure to dig two graves… Revenge was the only thing I had going for me. It kept me awake at night and drove me into desperate situations in dive bars across...
-
Man In The Water
Jon HillAn attempted murder. A missing spouse. And an international conspiracy that could change the world. Jack Green has always been skeptical of socalled facts. Though he's forced ...
-
Dracula
Bram StokerAn Apple Books Classic edition. Few characters have seized readers’ imaginations quite like Count Dracula of Transylvania, the hero of Bram Stoker’s classic. The 1897 novel put vam...
-
Tempting the King
Jessa YorkAn escaped Mafia Queen, hiding from her past. A Mafia King who wants to claim her… Giselle They think I'm lostbut I know better. I can never be found. The path I've creat...
-
Meditations
Emperor of Rome Marcus AureliusMeditations is a series of personal writings by Marcus Aurelius, Roman Emperor 161–180 CE, setting forth his ideas on Stoic philosophy.
-
Just Me
Lexy TimmsWe all need somewhere where we feel safe… After leaving her abusive husband, Katherine Marshall is out on her own for the first time. She's hopped from city to city to avoid t...
-
A Green Kind of Witch
Sierra CrossCinderella crossed with Mean Girls.   That's what Hazel's daily existence feels like. Born to a family of elegant yet shallow Beige Witches, seventeen y...