Mengetahui dan Mengenal Bells Palsy Bells Palsy adalah penyakit saraf yang mengenai saraf fasialis (wajah), menyebabkan kelumpuhan otot-otot salah satu sisi wajah, sehingga wajah menjadi asimetris, karena salah satu sisi wajah tampak melorot/ mencong. Hanya salah satu sisi wajah penderita saja yang dapat tersenyum, dan selain itu, hanya satu mata saja yang dapat menutup dengan sempurna.Penyebab dari Bells Palsy belum diketahui secara pasti, namun beberapa sumber menyebutkan bahwa penyebab Bells Palsy adalah proses peradangan saraf yang mengontrol otot-otot salah satu sisi wajah. Ada pula yang mengatakan bahwa kerusakan saraf ini merupakan akibat dari infeksi virus.Bells Palsy dapat terjadi pada semua golongan usia, baik pria maupun wanita. Pada kebanyakan orang, kelumpuhan saraf tersebut bersifat sementara, yakni selama beberapa hari hingga beberapa minggu, serta dapat kembali pulih setelah kurang lebih 6 bulan. Walaupun demikian, ada juga beberapa kasus dimana kelumpuhan saraf wajah tersebut terjadi secara permanen seumur hidup.GejalaGejala Bells Palsy secara umum: Terjadi secara tiba-tiba, berupa kelumpuhan ringan sampai total pada salah satu sisi wajah, menyebabkan pasien sulit tersenyum atau menutup salah satu kelopak mata Wajah melorot menjadikan wajah sulit berekspresi Dapat terjadi rasa nyeri di sekitar rahang atau di belakang telinga pada salah satu sisi wajah yang terpengaruh Sensitivitas terhadap suara akan meningkat pada sisi wajah yang terpengaruh Kadang timbul nyeri kepala Penurunan kemampuan indera pengecap pada sisi yang lumpuh Penurunan jumlah air mata dan liur yang diproduksi pada sisi yang terkena Pada beberapa kasus, Bells Palsy dapat mempengaruhi saraf kedua sisi wajah, walaupun hal tersebut jarang terjadiSegera Cari PertolonganPertolongan medis diperlukan sesegera mungkin jika Anda mengalami kelumpuhan apapun, karena bisa saja yang terjadi adalah kasus stroke. Perlu diingat, Bells Palsy bukan disebabkan oleh stroke. Segera temui dokter jika Anda mengalami kelemahan pada wajah, atau jika salah satu sisi wajah terlihat melorot tiba-tiba.Komplikasi Akibat Bells PalsyKomplikasi yang mungkin terjadi pada penderita Bells Palsy adalah: Kerusakan saraf wajah yang tidak dapat pulih kembali seperti semula Pertumbuhan saraf yang tidak sesuai dengan yang seharusnya sehingga menyebabkan pergerakan yang tidak terkontrol pada wajah Buta sebagian atau total akibat kekeringan pada mata yang tidak bisa menutup dan terjadinya kerusakan pada kornea mata yang keringPemeriksaanUmumnya dokter akan memeriksa wajah dan meminta pasien untuk melakukan gerakan seperti menutup mata, mengangkat alis, memperlihatkan gigi, dan mengerutkan wajah. Selain itu sebaiknya dilakukan pemeriksaan EMG untuk menentukan kerusakan saraf dan melihat seberapa parah saraf tersebut rusak, sekaligus menyingkirkan kemungkinan penyakit lain yang juga dapat menyebabkan kelemahan pada wajah, seperti stroke, infeksi, tumor, dll. Pasien juga disarankan melakukan pemeriksaan radiologi, seperti CT Scan atau MRI kepala untuk menyingkirkan penyebab kelumpuhan yang mungkin terjadi.Penanganan yang biasa dilakukan adalah melalui obat-obatan dan fisioterapi. Meskipun bisa sembuh dalam kurun waktu tertentu, dengan bantuan obat dan fisioterapi diharapkan proses pemulihan dapat berlangsung lebih cepat.

Bells palsy is a sudden or rapid onset facial paralysis or weakness which develops over hours to a day or two. If the face takes longer than 2-3 days to become paralyzed, the disorder is probably not Bells palsy and other causes need to be considered. Bells palsy, was formerly thought to be idiopathic or without known cause. The consensus now, is that it is a viral neuritis or viral inflammation of the nerve that makes the face to smile. The viruses which can cause viral neuritis likely represent what are called neurotropic or nerve-loving organisms. Among the viruses known to be able to cause acute facial paralysis are Herpes Simplex the fever blister-genital Herpes virus, and Herpes Zoster, the chicken pox-shingles virus. Lyme disease, which is not a virus but a spirochete bacterium, can cause a quite similar disorder. Bells palsy is thought to be primarily Herpes Simplex related.The incidence of Bells palsy is generally about 20-22 per 100,000 population per year. The rate is highest in pregnant women, a bit over 40 per 100,000 pregnant women per year. The lowest incidence is in children and the best prognosis is in children. About 70% of folks who get Bells palsy develop a rapid onset, single sided facial paralysis. About 30% develop diffuse facial weakness on a single side which usually recovers completely within 1-3 weeks. Rarely, does acute viral facial paralysis develop on both sides simultaneously. For a bit over 70%, recovery is back to normal within a month. About half of those who take longer than a month to recover return to completely normal ,and about half have at least some permanent side effects, but, generally, less than 5% have readily noticeable long term facial weakness with other signs of dysfunctional movement. The poorest prognosis applies to the elderly and long term diabetics but all experience at least some recovery of function, almost always quite functional. The repeat facial palsy rate, same or opposite side, is about 6-7% and about 7-10% have a family history of a similar problem. If a repeat Bells palsy occurs, the odds of a third recurrence nearly doubles to about 12-15% and if a third palsy occurs, the odds of a fourth palsy exceeds a 20% risk.Most Bells palsy patients, have some same-side ear or facial pain with onset of the facial palsy but it may precede the onset of the palsy by up to a few days or may develop after the single-sided facial paralysis. Other associated symptoms include dry eye, eye irritation, blurry vision, taste disturbance, vague numbness just in front of the ear, ringing in the ear, some dizziness or vertigo, and, relatively rarely, hearing loss.Quite similar to Bells palsy is Ramsay Hunt syndrome or shingles related facial palsy. Like Bells palsy, the facial paralysis onset is rapid but it is typically preceded by painful blisters on the outer ear or on the ear canal back wall. Pain with Ramsay Hunt syndrome is typical of shingles and may precede the paralysis by up to a week and rarely occurs without blisters (sine vesicles). Untreated, the prognosis for Ramsay Hunt syndrome is much worse than for Bells palsy with well over 50% suffering from permanent residual weakness and some having a permanently complete paralysis on one side of the face. Aggressive, early treatment lowers the bad-outcome risk...see treatment below.Mechanism of the Palsy:The mechanism by which the face becomes paralyzed in Bells Palsy involves several factors. First, the causative virus must exist within the nerve that makes the face to smile, the facial nerve. For the virus to get to the nerve, the person likely will have contracted the virus from another person by sharing forks or other food implements or by other intimate contact with a person who was actively shedding virus. It is not generally contagious by simply being in the same room. From initial contact, the virus multiplies and causes at least a localized skin or mucous membrane infection. In some folks, the virus may spread via the blood stream or through nerves to infect nerves at distant parts of the body. From that initial infection, the body develops immunity which generally keeps the virus under control or dormant but the immune system is generally not capable of ridding all of the nerve cells of dormant virus. Recent research suggests that immunologic mechanisms exist which do suppress the virus living within the nerve cells. Later, something happens which appears to ratchet back these immunologic control systems, allowing the dormant virus to replicate and make the nerve sick.A sick nerve becomes dysfunctional and swells. In the case of the facial nerve, an additional factor is the bony channel which it runs through to get to the face. As the facial nerve exits the brainstem, it is adjacent to the hearing and balance nerve as well as one of the taste nerves, the nervus intermedius. The latter is also the nerve which controls nose moisture and formation of tears for the eye. Where these nerves are together in the internal ear canal, they float in cerebrospinal fluid. At the lateral end of the internal ear canal, the hearing and balance nerves enter the inner ear, but the facial nerve and the nervus intermedius join together to course just above the inner ear. At this location, there are at least two nerve junction and cell body or ganglion areas. The one considered most likely the source of critical swelling is the ganglion of the meatal foramen. In this region of the facial nerve, a leathery brain covering wraps the nerve pretty tightly to prevent spinal fluid from leaking out along the nerve. The tightly wrapped nerve then enters the bony nerve canal which is barely large enough for a not-swollen nerve. Anatomic research suggests that the tighter this bony canal is, the less it allows a nerve to swell without compromising its function. In children, the bony canal is larger relative to nerve size than in adults. In some adults, the nerve just barely fits into the bony canal, leaving no room to tolerate nerve swelling during a viral nerve inflammation. Thus, in order to get Bells palsy, one must already have virus in the nerve, one must suffer some event that lowers immunity, and one must have anatomy which is too tight to allow the nerve to swell. In pregnancy, the natural tendency to retain fluid makes this situation more difficult.Sorting out the diagnosis:Bells palsy is not a stroke. Strokes rarely look quite like a Bells palsy. Generally, in Bells palsy, not just the lower to mid face is paralyzed on one side, but the forehead is as well. In a stroke-associated facial palsy, the forehead generally is still active. This is called forehead sparing. Such a stroke comes from the opposite side of the brain. The sparing occurs because some forehead nerve fibers come from the same side of the brain or do not crossover. It is theoretically possible for a rare tiny stroke in the brainstem to cause isolated facial palsy which looks exactly like Bells palsy but associated features such as pain would likely be quite different.Lyme disease, caused by the spirochete bacteria, Borrelia Borgdoferi, can cause facial paralysis that looks indistinguishable from viral Bells palsy, other than that pain seems less likely. This particular organism is transmitted by the deer tick and is not endemic or does not exist in mid to South Florida. Along the entire Gulf of Mexico coastline, a Lone-Star Texas tick may transmit a similar spirochete capable of doing the same. The same blood tests for Lyme disease are positive in both cases but Lyme disease facial paralysis is quite rare in those who have not been out of Florida in the spring to early summer when the nymph deer ticks are most likely to infect folks. In Lyme disease, the facial paralysis may occur at virtually any future date, but generally does so within a few seasons of the initial contact. Facial palsy from Lyme disease appears more likely to affect both sides of the face and is more common in children.Many other disease processes can cause facial paralysis but the vast majority of such palsies develop more slowly than does Bells palsy. Among the more critical to recognize are ear infection related facial palsy. The latter is more common in children more prone to acute middle ear infection, but may also occur at any age with a specific ear problem called infected cholesteatoma. A variety of tumors can also cause facial paralysis, generally taking 4 or more days to develop. Another rare, sometimes relapsing problem is called Melkerson-Rosenthal syndrome. In this disorder, along with facial paralysis, the patient also develops a swollen, coated tongue at the onset of the Palsy and typically swelling in the face. This disorder may be recurring as well.Recovery:The natural history of straight forward Bells palsy, untreated, is that all, yes all, recover at least to some degree, regardless of age. The incidence of recovery back to normal is about 70%. Residual facial weakness that is noticeable, to at least a minimal degree, is present in about 20-25%. Moderate to very noticeable residual facial weakness is much less common, generally affecting under 5% of folks under age 60 but a bit more common in the elderly, diabetics, and those affected during pregnancy. When residual weakness accompanying facial recovery, facial dyscoordination is also commonly present. For example, an effort to smile may cause the eye to close. Attempting to pucker may cause the smile-fold (nasolabial fold) at the corner of the mouth to deepen and the eye to close. Synkinesis is the term for this type of dysfunction. Commonly, synkinesis is also accompanied by weaker forehead recovery, as well as decreased ability to raise the eyebrow and a degree of eye dryness. With synkinesis, some develop large tears called crocodile tears while eating or talking as some of the nerve fibers intended for the facial muscles or the saliva glands in or below the mouth may be redirected to the eye tear gland.The natural rate of recovery generally starts within 3 weeks in 70-85% of patients. The longer it takes for recovery to begin, the more likely are the problems like incomplete recovery and synkinesis. Recovery always starts no later than 6 months after onset. If recovery has not started by 6 months, the problem is not Bells palsy. Recovery is at first subtle, but the rate of improvement speeds up and is generally complete within days to a very few weeks if it starts early. The longer the recovery waits to start, the slower the recovery, but the vast majority of recovery happens within 3 months of the first signs of recovery. Some additional improvement may occur over an additional 3 months, perhaps as long as a year or more.Risks related to facial palsy: The major risk during facial paralysis is failure to lubricate or protect the eye with eyelid closure. Lack of tears and failure of eyelid closure may lead to corneal abrasions which can lead to permanently impaired vision. Sometimes, a corneal transplant can repair the vision but not always. Thus, protecting the eye is the most important aspect of managing a facial paralysis.Treatment:In Bells palsy, appropriately aggressive treatment involves use of high dose steroids, generally 1 milligram of prednisone per kilogram of body weight, daily. Quite a number of research papers support the opinion that early steroids decrease pain and lessen the risk of major residual facial palsy. But, remember that at least 70% recover to normal with no treatment and only about 10-15% have moderate to major residual impaired dysfunction with no treatment. It appears that the severity of residual dysfunction can be lowered by early, high dose steroids. The earlier that they are started, the better, ideally within hours of disease onset. The natural tissue disease process causes the swelling to abate within about two weeks. Starting steroids that late is of doubtful benefit and continuing high dose steroids longer than 12-14 days after onset is probably not beneficial.Some propose adding antiviral drugs at the initiation of steroids for Bells palsy. At most, a small additional benefit accrues with the addition of drugs like valacyclovir (Valtrex) or its cousins, acyclovir or famciclovir. While the evidence that steroids are beneficial is fairly strong, the evidence that anti-Herpes medicine is helpful is less convincing, but it is common practice. However, in the case of Ramsay Hunt syndrome, a facial palsy caused by the shingles-Zoster-chicken pox virus, the use of early, very high dose antiviral therapy is associated with a markedly improved outcome. Whereas the vast majority of Bells palsy patients have quite good recovery, poor quality facial recovery in Ramsay Hunt may occur in up to 80% of untreated persons. For Ramsay Hunt patients, the poor quality recovery rate can drop to quite similar to Bells palsy if they are treated early high dose steroids and high dose antiviral medication. In Ramsay Hunt syndrome, a troubling but quite infrequent complication is permanent complete single-sided facial paralysis. Preventing it with early aggressive medication is strongly encouraged. Unlike Bells palsy, the swollen tissue takes longer to subside, perhaps longer than 20-21 days, and the permanent nerve injury risk is higher.Surgery may be appropriate for a very limited number of folks who develop acute single-sided facial palsy. When the facial paralysis is severe, with no apparent motion, an electromyogram or electroneurogram electrically stimulates the nerve. If the nerve remains able to be stimulated at four or more days after onset, the probability of spontaneous high quality recovery of function without surgery is very high. When the ability to stimulate the nerve is more than 95% lost and no voluntary effort is recordable electrically, the odds of a poor quality recovery may exceed 50%. That testing is only valid if done between days 4 and 14. If at that point, urgent surgery removes the bone that is constricting a swollen nerve, the odds of high quality recovery improve to over 85% but the surgery must be done before the 15th day, if it is to be done at all. Doing the surgery later than this does not improve outcome and we prefer if at all possible to do the surgery before day 12-13 as the probability of benefit seems to decline beyond day 12 of the illness.Surgery:The surgery is called middle fossa facial nerve decompression. An incision is made from just in front of the ear directed upwards about 7-8 centimeters, or about 3-3.5 inches, exposing the underlying muscle. After mobilizing the muscle, the surgeon opens a window in the skull measuring about 3.5 centimeters square, exposing the leathery brain covering called dura mater. The surgery purposely stays out of the brain but does elevate the dura from where it overlies the top of the inner ear. Between the hearing and balance organ, the surgeon uses fine diamond burrs to sand away a channel in bone until the internal ear canal and the facial nerve are reached. Opening the bony canal of the facial nerve removes the tight constriction of bone around the inflamed nerve, allowing it to swell. A swollen nerve constricted in the tight bony canal loses its blood supply. By allowing it to swell, the blood circulation to the nerve recovers and the nerve is more likely to recover. The surgeon replaces the bone window in the skull with titanium plates and puts the muscle back. After closing the skin, the surgical team covers the incision with a dressing and sends the patient to recover overnight in the intensive care unit. Most patients go home after 1-2 days in the hospital. Risks include leakage of spinal fluid, decreased hearing, dizziness, and bleeding inside the head, all pretty rare. Infection is rare and failure of the facial nerve to recover is possible.For those who have recurring Bells palsies, middle fossa facial nerve decompression stops the recurring paralysis but not the recurring viral nerve infections. The recurring viral nerve infections are thought to be equal to recurring fever blisters or recurring shingles. The surgery allows space for the nerve to swell when the virus affects it, typically not bad enough to cause paralysis. Our local experience with quite a few of these patients which indicates that long term antiviral medication prevents the recurring palsies, without the need for surgery. However, long term antivirals are preventive and do not have more than a minor affect on recovery of the acute palsy. ie., these medications do not remove the potential need to decompress a nerve that meets decompression criteria.Treatment of Ramsay Hunt:For those with Ramsay Hunt syndrome, (shingles associated facial paralysis), our preference is early, very high dose antiviral medication and high dose steroids, continuing both until at least the 21st day after disorder onset. While the published literature suggests that middle fossa facial nerve decompression is effective for Bells palsy, the question of adequacy of benefit from surgery has not been proven in Ramsay Hunt syndrome. Nevertheless, since nerve swelling seems to be the in-common issue that causes poor quality recovery, many think surgery is indicated for severe Ramsay Hunt facial paralysis.In summary, Bells palsy can be a quite disfiguring disorder, but fortunately, for the vast majority, high quality recovery occurs within weeks. For those with delayed recovery, satisfactory function is highly likely but some degree of impairment, generally not severe, may remain. For those whose prognosis for a poor quality recovery seems high, surgery is a possibility. When facial paralysis features do not fit well with the classic features of Bells palsy or Ramsay Hunt syndrome, a careful evaluation for other causes is essential, as other very serious issues may exist for which other specific management is appropriate.

Amyotrophic lateral sclerosis (ALS) adalah penyakit neurologis serius yang menyebabkan kelemahan otot, kecacatan, dan akhirnya kematian.ALS sering disebut pula sebagai penyakit Lou Gehrig, diambil dari nama pemain baseball terkenal AS yang didiagnosis penyakit ini pada tahun 1939.Di seluruh dunia, diperkirakan ALS terjadi pada 1 hingga 3 orang per 100.000 populasi.Sebagian besar kasus ALS belum diketahui penyebab pastinya dengan sebagian kecil diakibatkan oleh faktor keturunan.ALS sering dimulai dengan sentakan otot dan kelemahan di lengan atau kaki, serta bicara yang mendadak tidak lancar.Akhirnya, ALS mempengaruhi kemampuan seseorang untuk mengontrol otot-otot yang diperlukan untuk bergerak, berbicara, makan, dan bernapas.GejalaTanda awal dan gejala ALS meliputi: Kesulitan mengangkat bagian depan telapak kaki dan jari kaki Kelemahan di kaki, pergelangan kaki, atau telapak kaki Kelemahan pada tangan Kesulitan berbicara atau kesulitan menelan Kram otot dan berkedut di lengan, bahu dan lidahPenyakit ini sering dimulai pada tangan atau kaki untuk kemudian menyebar ke bagian lain tubuh.Seiring meningkatnya keparahan, otot-otot menjadi semakin lemah sampai lumpuh yang akhirnya mempengaruhi kemampuan mengunyah, menelan, berbicara, dan bernapas.PenyebabALS dipicu oleh kematian sel-sel saraf yang mengendalikan gerakan otot sehingga membuat otot menjadi lemah dan lumpuh.Satu dari 10 kasus ALS disebabkan faktor genetik, sedangkan sisanya belum diketahui pasti penyebabnya.Para peneliti sedang mempelajari beberapa kemungkinan penyebab ALS, termasuk:1. Mutasi genBerbagai mutasi genetik dapat memicu ALS. Tidak ada perbedaan signifikan antara ALS akibat genetik dengan ALS yang disebabkan faktor lain.2. Ketidakseimbangan kimiaOrang yang mengalami ALS biasanya memiliki kadar glutamat lebih tinggi dari normal.Glutamate merupakan bahan kimia pembawa pesan dalam otak dan sistem saraf.Terlalu banyak glutamat diketahui menjadi racun bagi beberapa sel-sel saraf.3. Respon kekebalan yang tidak terkendaliKekebalan tubuh seseorang mungkin mulai menyerang sel-sel tubuh normal. Kondisi ini diduga bisa memicu proses yang mengakibatkan ALS.4. Kesalahan penanganan proteinTerdapat bukti bahwa kesalahan penanganan protein dalam sel-sel saraf dapat menyebabkan akumulasi bertahap bentuk abnormal protein dalam sel yang memicu kematian sel-sel saraf.Faktor RisikoFaktor risiko yang memperbesar kemungkinan seseorang mengalami ALS meliputi:1. KeturunanSekitar 10 persen orang yang mengalami ALS mewarisinya dari orangtua mereka.Anak yang memiliki orang tua ALS memiliki peluang 50-50 mengalami penyakit yang sama.2. UsiaALS paling umum terjadi pada orang berusia 40 tahun hingga 60 tahun.3. Jenis kelaminSebelum usia 65 tahun, pria memiliki peluang sedikit lebih besar dibandingkan wanita mengalami ALS.Perbedaan ini akan lenyap pada rentang usia di atas 70 tahun.Penelitian menunjukkan bahwa ALS bisa dipicu oleh faktor lingkungan tertentu pada orang yang sudah membawa predisposisi genetik untuk penyakit ini.Faktor lingkungan yang memperbesar risiko mengalami ALS meliputi:1. MerokokMerokok meningkatkan risiko seseorang mengalami ALS hampir dua kali lipat dibandingkan orang yang tidak merokok.2. Paparan timbalBeberapa bukti menunjukkan bahwa paparan timbal di tempat kerja dapat dikaitkan dengan perkembangan ALS.3. Dinas militerStudi terbaru menunjukkan bahwa orang yang bertugas di militer mengalami risiko lebih tinggi mengembangkan ALS.Tidak diketahui pasti alasan di balik penemuan ini, tetapi dugaan berkisar pada paparan logam atau kimia tertentu, luka traumatis, infeksi virus, dan aktivitas fisik yang intens