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Cricopharyngeal spasms occur in the cricopharyngeus muscle of the pharynx. These spasms are frequently misunderstood by the patient to be cancer due to the 'lump in the throat' feeling (Globus pharyngis) that is symptomatic of this syndrome. In practice, real lumps in the throat, such as a thyroid cancer, are generally not felt until they impede ingestion of food. This is one of the reasons that a cancer can get so big before it is discovered. In contrast, a cricopharyngeal spasm is an uncomfortable but harmless and temporary disorder.
Globus pharyngis (also known as globus sensation, globus or, somewhat outdatedly, globus hystericus, commonly referred to as having a "lump in one's throat"), is the persistent sensation of having phlegm, a pill or some other sort of obstruction in the throat when there is none. Swallowing can be performed normally, so it is not a true case of dysphagia, but it can become quite irritating. One may also feel mild chest pain or even severe pain with a clicking sensation when swallowing.
Causes include stress and anxiety. Other causes are not yet clear. In some cases, eating certain foods may bring on acute spasms, in susceptible individuals. Peanuts, pumpkin seeds and other nuts may trigger these spasms.
The "lump in the throat" sensation that characterizes globus pharyngis is often caused by inflammation of one or more parts of the throat, such as the larynx or hypopharynx, due to cricopharyngeal spasm, gastroesophageal reflux (GERD), laryngopharyngeal reflux or esophageal versatility.
In some cases the cause is unknown and symptoms may be attributed to a cause "i.e." a somatoform or anxiety disorder. It has been recognised as a symptom of depression, which responds to anti-depressive treatment.
Differential diagnosis must be made from Eagle syndrome which uses the patient's description of "something caught in my throat" as a diagnostic tool. Eagle syndrome is an elongation of the styloid process causing irritation to nerves and muscles in the region resulting in a number of unusual symptoms.
The results of recent studies have strongly suggested that GERD is a major cause of globus, though this remains under considerable debate.
A less common cause, distinguished by a "lump in the throat" accompanied with clicking sensation and considerable pain when swallowing, may be due to thyroid-cartilage rubbing against anomalous asymmetrical laryngeal anatomy "e.g." the superior cornu abrading against the thyroid lamina, surgically trimming the offending thyroid-cartilage provides immediate relief in all cases. However this cause is frequently misdiagnosed, despite requiring a simple clinical examination involving careful palpation of the neck side to side which elicits the same click sensation (laryngeal crepitus) and pain as when swallowing, most cases are due to prior trauma to the neck. High resolution computed tomographic (CT) or MRI scan of the larynx is usually required to fully understand the anomalous laryngeal anatomy. Anterior displacement of the thyroid ala on the affected side while swallowing can help resolve symptoms.
Trismus, also called lockjaw, is reduced opening of the jaws (limited jaw range of motion). It may be caused by spasm of the muscles of mastication or a variety of other causes. Temporary trismus occurs much more frequently than permanent trismus. It is known to interfere with eating, speaking, and maintaining proper oral hygiene. This interference, specifically with the patient's ability to swallow properly, results in an increased risk of aspiration. In some instances, trismus presents with altered facial appearance. The condition may be distressing and painful for the patient. Examination and treatments requiring access to the oral cavity can be limited, or in some cases impossible, due to the nature of the condition itself.
Atypical facial pain (AFP) is a type of chronic facial pain which does not fulfill any other diagnosis. There is no consensus as to a globally accepted definition, and there is even controversy as to whether the term should be continued to be used. Both the International Headache Society (IHS) and the International Association for the Study of Pain (IASP) have adopted the term persistent idiopathic facial pain (PIFP) to replace AFP. In the 2nd Edition of the International Classification of Headache Disorders (ICHD-2), PIFP is defined as "persistent facial pain that does not have the characteristics of the cranial neuralgias [...] and is not attributed to another disorder." However, the term AFP continues to be used by the World Health Organization's 10th revision of the International Statistical Classification of Diseases and Related Health Problems and remains in general use by clinicians to refer to chronic facial pain that does not meet any diagnostic criteria and does not respond to most treatments.
The main features of AFP are: no objective signs, negative results with all investigations/ tests, no obvious explanation for the cause of the pain, and a poor response to attempted treatments. AFP has been described variably as a medically unexplained symptom, a diagnosis of exclusion, a psychogenic cause of pain (e.g. a manifestation of somatoform disorder), and as a neuropathy. AFP is usually burning and continuous in nature, and may last for many years. Depression and anxiety are often associated with AFP, which are either described as a contributing cause of the pain, or the emotional consequences of suffering with unrelieved, chronic pain. For unknown reasons, AFP is significantly more common in middle aged or elderly people, and in females.
Atypical odontalgia (AO) is very similar in many respects to AFP, with some sources treating them as the same entity, and others describing the former as a sub-type of AFP. Generally, the term AO may be used where the pain is confined to the teeth or gums, and AFP when the pain involves other parts of the face. As with AFP, there is a similar lack of standardization of terms and no consensus regarding a globally accepted definition surrounding AO. Generally definitions of AO state that it is pain with no demonstrable cause which is perceived to be coming from a tooth or multiple teeth, and is not relieved by standard treatments to alleviate dental pain.
Depending upon the exact presentation of atypical facial pain and atypical odontalgia, it could be considered as craniofacial pain or orofacial pain. It has been suggested that, in truth, AFP and AO are umbrella terms for a heterogenous group of misdiagnosed or not yet fully understood conditions, and they are unlikely to each represent a single, discrete condition.
Neurological abnormalities are common. Roughly 45% of people with Parry–Romberg syndrome are also afflicted with trigeminal neuralgia (severe pain in the tissues supplied by the ipsilateral trigeminal nerve, including the forehead, eye, cheek, nose, mouth and jaw) and/or migraine (severe headaches that may be accompanied by visual abnormalities, nausea and vomiting).
10% of affected individuals develop a seizure disorder as part of the disease. The seizures are typically Jacksonian in nature (characterized by rapid spasms of a muscle group that subsequently spread to adjacent muscles) and occur on the side contralateral to the affected side of the face. Half of these cases are associated with abnormalities in both the gray and white matter of the brain—usually ipsilateral but sometimes contralateral—that are detectable on magnetic resonance imaging (MRI) scan.
AFP and AO can be difficult to diagnose, and are often misdiagnosed with resultant inappropriate attempts of treatment, e.g. root canal therapy which may at best have only a temporary benefit, or at worst lead to an increase in the pain. Excluding an organic cause for the pain is the most important part of the diagnosis. Odontogenic pain should especially be ruled out, since this accounts for over 95% of cases of orofacial pain.
There is considerable symptom overlap between atypical facial pain and temporomandibular joint dysfunction.
The diagnosis of facial pain generally is often multidisciplinary.
Enophthalmos (recession of the eyeball within the orbit) is the most common eye abnormality observed in Parry–Romberg syndrome. It is caused by a loss of subcutaneous tissue around the orbit. Other common findings include drooping of the eyelid (ptosis), constriction of the pupil (miosis), redness of the conjunctiva, and decreased sweating (anhidrosis) of the affected side of the face. Collectively, these signs are referred to as Horner's syndrome. Other ocular abnormalities include ophthalmoplegia (paralysis of one or more of the extraocular muscles) and other types of strabismus, uveitis, and heterochromia of the iris.
Trismus is defined as difficulty in opening the mouth due to a muscle spasm resulting from a disturbance in the trigeminal nerve, however it can also refer to limited mouth opening of any cause. Another definition of trismus is simply a limitation of movement. Historically and commonly, the term "lock jaw" was sometimes used as a synonym for both trismus and tetanus.
Normal mouth-opening ranges from 35 to 45 mm. Males usually have slightly greater mouth opening than females. (40–60 mm, average of 35 mm). The Normal Lateral movement is 8-12mm, and normal protrusive movement is approximately 10mm. Some have distinguished mild trismus as 20–30 mm interincisal opening, moderate as 10–20 mm and severe as less than 10 mm.
Trismus is derived from the Irish word "trismos" meaning "a scream; a grinding, rasping or gnashing"
Palatal myoclonus is a rapid spasm of the palatal (roof of the mouth) muscles, which results in clicking or popping in the ear. Chronic clonus is often due to lesions of the central tegmental tract (which connects the red nucleus to the ipsilateral inferior olivary nucleus). Uniquely, the clicking noise does not subside when the patient sleeps.
When associated with eye movements, it is known as "oculopalatal myoclonus"
VBD
- Hemifacial spasm
- Paresis
- Trigeminal neuralgia
ICD
- Progressive visual field defect
It most often occurs in the middle of the night and lasts from seconds to minutes, an indicator for the differential diagnosis of levator ani syndrome, which presents as pain and aching lasting twenty minutes or longer. In a study published in 2007 involving 1809 patients, the attacks occurred in the daytime (33 per cent) as well as at night (33 per cent) and the average number of attacks was 13. Onset can be in childhood; however, in multiple studies the average age of onset was 45. Many studies showed that women are affected more commonly than men. This can be at least partly explained by men's reluctance to seek medical advice concerning such a delicate case as rectal pain.
During an episode, the patient feels spasm-like, sometimes excruciating, pain in the anus, often misinterpreted as a need to defecate. The pain must arise de novo, that is in absence of clear cause. As such, pain associated with penetrative anal intercourse, trauma or rectal foreign body insertion preclude a diagnosis of proctalgia fugax. Simultaneous stimulation of the local autonomic system can cause erection in males. In some people, twinges sometimes occur shortly after orgasm. Because of the high incidence of internal anal sphincter thickening with the disorder, it is thought to be a disorder of the internal anal sphincter or that it is a neuralgia of pudendal nerves. It is recurrent and there is also no known cure. However, some studies show effective use of botulinum toxin, pudendal nerve block, and calcium channel blockers. It is not known to be linked to any disease process and data on the number of people afflicted vary, but prevalence may be as high as 8–18%. It is thought that only 17–20% of sufferers consult a physician, so obtaining accurate data on occurrence presents a challenge.
The pain episode subsides by itself as the spasm disappears on its own, but may reoccur.
The first sign of hemifacial spasm is typically muscle movement in the patient's eyelid and around the eye. It can vary in intensity. The intermittent twitching of the eyelid, which can result in forced closure of the eye which gradually spreads to the muscles of the lower part of the face (Typical form- See Image). In atypical form the spasms start in the cheekbone area and spreads to the eyelid. Ultimately, all the muscles on that side are affected, nearly all the time. This sometimes causes the mouth to be pulled to the side. Experts have linked hemifacial spasm to facial nerve injury, Bell's palsy and tumors. Although the most frequent cause is a blood vessel pressing on the facial nerve at the spot where it leaves the patient's brain stem, sometimes there is no known cause. When the affected individual is younger than 40, doctors suspect an underlying cause such as multiple sclerosis.
Oromandibular Symptoms
- difficulty opening the mouth (trismus)
- clenching or grinding of the teeth (bruxism)
- spasms of jaw opening
- sideways deviation or protrusion of the jaw
- lip tightening and pursing
- drawing back (retraction) of the corners of the mouth
- deviation or protrusion of the tongue.
- jaw pain
- difficulties eating and drinking
- difficulties speaking (dysarthria)
Blepharospasm symptoms
- the first symptom to appear is an increased rate of blinking
- uncontrollable squinting/closing of eyes
- light sensitivity (photophobia)
- squinting/eyes closing during speech
- uncontrollable eyes closing shut (rare instances completely causing blindness)
In addition, in some patients, the dystonic spasms may sometimes be provoked by certain activities, such as talking, chewing, or biting. Particular activities or sensory tricks may sometimes temporarily alleviate OMD symptoms, including chewing gum, talking, placing a toothpick in the mouth, lightly touching the lips or chin, or applying pressure beneath the chin.
The main symptoms involve involuntary blinking and chin thrusting. Some patients may experience excessive tongue protrusion, squinting, light sensitivity, muddled speech, or uncontrollable contraction of the platysma muscle. Some Meige's patients also have "laryngeal dystonia" (spasms of the larynx). Blepharospasm may lead to embarrassment in social situations, and oromandibular dystonia can affect speech, making it difficult to carry on the simplest conversations. This can cause difficulty in both personal and professional contexts, and in some cases may cause patients to withdraw from social situations.
The condition tends to affect women more frequently than men.
Proctalgia fugax (a variant of levator ani syndrome) is a severe, episodic pain in the regions of the rectum and anus. It can be caused by cramp of the levator ani muscle, particularly in the pubococcygeal part.
Hemifacial spasm (HFS) is a rare neuromuscular disease characterized by irregular, involuntary muscle contractions (spasms) on one side (hemi-) of the face (-facial). The facial muscles are controlled by the facial nerve (seventh cranial nerve), which originates at the brainstem and exits the skull below the ear where it separates into five main branches.
This disease takes two forms: typical and atypical. In typical form, the twitching usually starts in the lower eyelid in orbicularis oculi muscle. As time progresses, it spreads to the whole lid, then to the orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area. The reverse process of twitching occurs in atypical hemifacial spasm; twitching starts in orbicularis oris muscle around the lips, and buccinator muscle in the cheekbone area in the lower face, then progresses up to the orbicularis oculi muscle in the eyelid as time progresses. The most common form is the typical form, and atypical form is only seen in about 2–3% of patients with hemifacial spasm. The incidence of hemifacial spasm is approximately 0.8 per 100,000 persons.
This disorder occurs in both men and women, although it affects middle-aged or elderly women more frequently. Hemifacial spasm is much more common in some Asian populations. It may be caused by a facial nerve injury, a tumor, or it may have no apparent cause. Individuals with spasm on both sides of the face are very rare.
French Professor Armand Trousseau (1801-1867) devised the trick (now known as the Trousseau sign of latent tetany) of occluding the brachial artery by squeezing, to trigger cramps in the fingers. Also, tetany can be demonstrated by tapping anterior to the ear, at the emergence of the facial nerve. A resultant twitch of the nose or lips suggests low calcium levels. This is now known as the Chvostek sign.
Tetany is characterized by contraction of distal muscles of the hands (carpal spasm with extension of interphalangeal joints and adduction and flexion of the metacarpophalangeal joints) and feet (pedal spasm) and is associated with tingling around the mouth and distally in the limbs.
EMG studies reveal single or often grouped motor unit discharges at low discharge frequency during tetany episodes.
Tetany or tetany seizure is a medical sign consisting of the involuntary contraction of muscles, which may be caused by disease or other conditions that increase the action potential frequency of muscle cells or the nerves that innervate them. Muscle cramps which are caused by the disease tetanus are not classified as tetany; rather, they are due to a lack of inhibition to the neurons that supply muscles.
Jactitation is an archaic medical term (derived, perhaps as a corruption, from "jactation", meaning a restless tossing and turning of the body, and derived itself from Latin "jactare" or "jacere", both meaning "to throw or hurl") referring to the involuntary spasm of a limb, muscle, or muscle group. This is sometimes seen in fever patients or other situations of physical distress, but may occur in healthy individuals in a hypnogogic state. This hypnagogic jactitation often occurs in the legs, and may occasion a short explanatory dream about stumbling or missing the bottom stair.
DES manifests as intermittent difficulty swallowing (dysphagia) for solid foods and liquids, and atypical chest pain. The chest pain may appear similar to cardiac chest pain (angina pectoris), so investigating the possible existence of heart disease is often indicated.
Hypomimia (masked facies, masking of facies), a medical sign, is a reduced degree of facial expression. It can be caused by motor impairment (for example, weakness or paralysis of the facial muscles), as in Parkinson's disease, or by other causes, such as psychological or psychiatric factors (for example, if a patient does not feel emotions and thus does not show any expression).
Persons receiving excessive Botox treatments, and thusly losing disproportionate facial expression features may be incorrectly identified as suffering hypomimia.
The term dolichoectasia means elongation and distension. It is used to characterize arteries throughout the human body which have shown significant deterioration of their tunica intima (and occasionally the tunica media), weakening the vessel walls and causing the artery to elongate and distend.
The main symptom is choking and difficulty or inability to breathe or speak, a feeling of suffocation, which may be followed by hypoxia-induced loss of consciousness. As the airway reopens, breathing may cause a high-pitched sound called stridor. The episode seldom lasts over a couple of minutes before breathing is back to normal.