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Onset of symptoms usually occur in early adulthood and is characterized by intention tremor, progressive ataxia, convulsions, and myoclonic epileptic jerks.
Tremors usually affect one extremity, primarily the upper limb, and eventually involve the entire voluntary motor system. Overall, the lower extremity is usually disturbed less often than the upper extremity.
Additional features of the syndrome include: an unsteady gait, seizures, muscular hypotonia, reduced muscular coordination, asthenia, adiadochokinesia and errors with estimating range, direction, and force of voluntary movements. Mental deterioration can occur, however it is rare.
Ramsay Hunt syndrome (RHS) type 1 is a rare, degenerative, neurological disorder characterized by myoclonus epilepsy, intention tremor, progressive ataxia and occasionally cognitive impairment
It has also been alternatively called "dyssynergia cerebellaris myoclonica", "dyssynergia cerebellaris progressiva", dentatorubral degeneration, or Ramsay Hunt cerebellar syndrome.
Cerebellar ataxia can occur as a result of many diseases and presents with symptoms of an inability to coordinate balance, gait, extremity and eye movements. Lesions to the cerebellum can cause dyssynergia, dysmetria, dysdiadochokinesia, dysarthria and ataxia of stance and gait. Deficits are observed with movements on the same side of the body as the lesion (ipsilateral). Clinicians often use visual observation of people performing motor tasks in order to look for signs of ataxia.
There are many causes of cerebellar ataxia including, among others, gluten ataxia, autoimmunity to Purkinje cells or other neural cells in the cerebellum, CNS vasculitis, multiple sclerosis, infection, bleeding, infarction, tumors, direct injury, toxins (e.g., alcohol), genetic disorders, and an association with statin use. Gluten ataxia accounts for 40% of all sporadic idiopathic ataxias and 15% of all ataxias.
Intention tremors are common among individuals with multiple sclerosis (MS). One common symptom of multiple sclerosis is ataxia, a lack of coordinated muscle movement caused by cerebellar lesions characteristic of multiple sclerosis. The disease often destroys physical and cognitive function of individuals.
Intention tremors can be a first sign of multiple sclerosis, since loss or deterioration of motor function and sensitivity are often one of the first symptoms of cerebellar lesions.
Intention tremors have a variety of other recorded causes as well. These include a variety of neurological disorders, such as stroke, alcoholism, alcohol withdrawal, peripheral neuropathy, Wilson's disease, Creutzfeldt–Jakob disease, Guillain–Barré syndrome and fragile X syndrome, as well as brain tumors, low blood sugar, hyperthyroidism, hypoparathyroidism, insulinoma, normal aging, and traumatic brain injury. Holmes tremor, a rubral or midbrain tremor, is another form of tremor that includes intention tremors, among other symptoms. This disease affects the proximal muscles of the head, shoulders, and neck. Tremors of this disease occur at frequencies of 2–4 Hz or more.
Intention tremor is also known to be associated with infections, West Nile virus, rubella, H. influenza, rabies, and varicella. A variety of poisons have been shown to cause intention tremor, including mercury, methyl bromide, and phosphine. In addition, vitamin deficiencies have been linked to intention tremor, especially deficiency in vitamin E. Pharmacological agents such as anti-arrhythmic drugs, anti-epileptic agents, benzodiazepine, cyclosporine, lithium, neuroleptics, and stimulants have been known to cause intention tremor. Some ordinary activities including ingesting too much caffeine, cigarettes, and alcohol, along with stress, anxiety, fear, anger and fatigue
have also been shown to cause intention tremor by negatively affecting the cerebellum, brainstem, or thalamus, as discussed in mechanisms.
The pathophysiology of the condition results from neuronal plasticity associated with bladder afferents and motor neurons innervating the external urethral sphincter. People with this condition generally experience daytime and night time wetting, urinary retention, and often have a history of urinary tract and bladder infections. Constipation and encopresis are often associated with this condition.
Intention tremor, also known as cerebellar tremor, is a dyskinetic disorder characterized by a broad, coarse, and low frequency (below 5 Hz) tremor. The amplitude of an intention tremor increases as an extremity approaches the endpoint of deliberate and visually guided movement (hence the name intention tremor). An intention tremor is usually perpendicular to the direction of movement. When experiencing an intention tremor, one often overshoots or undershoots their target, a condition known as dysmetria. Intention tremor is the result of dysfunction of the cerebellum, particularly on the same side as the tremor in the lateral zone, which controls visually guided movements. Depending on the location of cerebellar damage, these tremors can be either unilateral or bilateral.
A variety of causes have been discovered to date, including damage or degradation of the cerebellum due to neurodegenerative diseases, trauma, tumor, stroke, or toxicity. There is currently no established pharmacological treatment; however, some success has been seen using treatments designed for essential tremors.
Bladder sphincter dyssynergia (also known as detrusor sphincter dysynergia (DSD) (the ICS standard terminology agreed 1998) and neurogenic detrusor overactivity (NDO)) is a consequence of a neurological pathology such as spinal injury or multiple sclerosis. which disrupts central nervous system regulation of the micturition (urination) reflex resulting in dyscoordination of the detrusor muscles of the bladder and the male or female external urethral sphincter muscles. In normal lower urinary tract function, these two separate muscle structures act in synergistic coordination. But in this neurogenic disorder, the urethral sphincter muscle, instead of relaxing completely during voiding, dyssynergically contracts causing the flow to be interrupted and the bladder pressure to rise.
Symptoms include:
- Straining to pass fecal material
- Tenesmus (a feeling of incomplete evacuation)
- Feeling of anorectal obstruction/blockage
- Digital maneuvers needed to aid defecation
- Difficulty initiating and completing bowel movements
Anismus is classified as a functional defecation disorder. It is also a type of rectal outlet obstruction (a functional outlet obstruction). Where anismus causes constipation, it is an example of functional constipation. Some authors describe an "obstructed defecation syndrome", of which anismus is a cause.
The Rome classification subdivides functional defecation disorders into 3 types, however the symptoms the patient experiences are identical.
- Type I: paradoxical contraction of the pelvic floor muscles during attempted defecation
- Type II: inadequate propulsive forces during attempted defecation (inadequate defecatory propulsion)
- Type III: impaired relaxation with adequate propulsion
It can be seen from the above classification that many of the terms that have been used interchangeably with anismus are inappropriately specific and neglect the concept of impaired propulsion. Similarly, some of the definitions that have been offered are also too restrictive.
A prostatic stent is a stent used to keep open the male urethra and allow the passing of urine in cases of prostatic obstruction and lower urinary tract symptoms (LUTS). Prostatic obstruction is a common condition with a variety of causes. Benign prostatic hyperplasia (BPH) is the most common cause, but obstruction may also occur acutely after treatment for BPH such as transurethral needle ablation of the prostate (TUNA), transurethral resection of the prostate (TURP), transurethral microwave thermotherapy (TUMT), prostate cancer or after radiation therapy.
There are two types of prostatic stent: temporary and permanent.
Although a permanent prostatic stent is not a medical treatment, it falls under the classification of a surgical procedure. Placement of a permanent prostatic stent is carried out as an outpatient treatment under local, topical or spinal anesthesia and usually takes about 15–30 minutes.
A temporary prostatic stent can be inserted in a similar manner to a Foley catheter, requiring only topical anesthesia.
Depending on the level of obstruction, bowel obstruction can present with abdominal pain, swollen abdomen, abdominal distension, vomiting, fecal vomiting, and constipation.
Bowel obstruction may be complicated by dehydration and electrolyte abnormalities due to vomiting; respiratory compromise from pressure on the diaphragm by a distended abdomen, or aspiration of vomitus; bowel ischemia or perforation from prolonged distension or pressure from a foreign body.
In small bowel obstruction, the pain tends to be colicky (cramping and intermittent) in nature, with spasms lasting a few minutes. The pain tends to be central and mid-abdominal. Vomiting may occur before constipation.
In large bowel obstruction, the pain is felt lower in the abdomen and the spasms last longer. Constipation occurs earlier and vomiting may be less prominent. Proximal obstruction of the large bowel may present as small bowel obstruction.
Causes of small bowel obstruction include:
- Adhesions from previous abdominal surgery (most common cause)
- Barbed sutures.
- Pseudoobstruction
- Hernias containing bowel
- Crohn's disease causing adhesions or inflammatory strictures
- Neoplasms, benign or malignant
- Intussusception
- Volvulus
- Superior mesenteric artery syndrome, a compression of the duodenum by the superior mesenteric artery and the abdominal aorta
- Ischemic strictures
- Foreign bodies (e.g. gallstones in gallstone ileus, swallowed objects)
- Intestinal atresia
After abdominal surgery, the incidence of small bowel obstruction from any cause is 9%. In those where the cause of the obstruction was clear, adhesions are the single most common cause (more than half).