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Thursday, May 14, 2009

Multiple sclerosis signs and symptoms

Multiple sclerosis can cause a variety of symptoms, including paranoid delusions, changes in sensation (hypoesthesia), muscle weakness, abnormal muscle spasms, or difficulty to move; difficulties with coordination and balance; problems in speech (Dysarthria) or swallowing (Dysphagia), visual problems (Nystagmus, optic neuritis, or diplopia), fatigue and acute or chronic pain syndromes, bladder and bowel difficulties, cognitive impairment, or emotional symptomatology (mainly major depression). The main clinical measure of disability progression and severity of the symptoms is the Expanded Disability Status Scale or EDSS.

The initial attacks are often transient, mild (or asymptomatic), and self-limited. They often do not prompt a health care visit and sometimes are only identified in retrospect once the diagnosis has been made based on further attacks. The most common initial symptoms reported are: changes in sensation in the arms, legs or face (33%), complete or partial vision loss (optic neuritis) (20%), weakness (13%), double vision (7%), unsteadiness when walking (5%), and balance problems (3%); but many rare initial symptoms have been reported such as aphasia or psychosis. Fifteen percent of individuals have multiple symptoms when they first seek medical attention. For some people the initial MS attack is preceded by infection, trauma, or strenuous physical effort.


Bladder

Bladder problems (See also urinary system and urination) appear in 70-80% of MS patients and they have an important effect both in hygiene habits and social activity. However bladder problems are usually related with high levels of disability and pyramidal signs in lower limbs.

The most common problems are an increase of frequency and urgency (incontinence) but difficulties to begin urination, hesitation, leaking, retention and sensation of incomplete urination also appear. When there is retention secondary urinary infections are common.

There are many cortical and subcortical structures implicated in micturition. Accordingly; MS lesions in different central nervous system structures can cause these kind of symptoms.

Treatment objectives are alleviation of symptoms of urinary dysfunction, treatment of urinary infections, reduction of complicating factors and preservation of renal function.Treatments can be classified in two main subtypes: pharmacological and non pharmacological. Pharmacological treatments vary greatly depending on the origin or type of dysfunction; however some examples of the medications used are: alfuzosin for retention, trospium and flavoxate for urgency and incontinency, or desmopressin for nocturia. Non pharmacological treatments involve the use of pelvic floor muscle training, stimulation, biofeedback, pessaries, bladder retraining, and sometimes intermittent catheterization.


Cognitive

Cognitive impairments are common. Neuropsychological studies suggest that 40 to 60 percent of patients have cognitive deficits; with the lowest percentages usually from community-based studies and the highest ones from hospital-based.

Cognitive impairment, sometimes referred to as brain fog, is already present in the beginnings of the disease. Even in probable MS (after the first attack but before a second confirmatory one) up to 50% of patients have mild impairment.

Some of the most common declines are in recent memory, attention, processing speed, visual-spatial abilities and executive functions. Other cognitive-related symptoms are emotional instability, and fatigue, including purely neurological fatigue. The cognitive impairments in MS are usually mild; and only in 5% of patients can we speak of dementia. Nevertheless they are related with unemployment and reduced social interactions. They are also related with driving difficulties.

Neurocognitive testing is important for determining the extent of cognitive deficits. Neuropsychological stimulation may help to reverse or decrease the cognitive defects although its management relies on lifestyle strategies.Interferons have demonstrated that can help to reduce cognitive limitations in multiple sclerosis. Anticholinesterase drugs such as donepezil commonly used in alzheimer disease; although not approved yet for multiple sclerosis; have also shown efficacy in different clinical trials.

There have also been some studies looking at certain ADHD stimulants, like Ritalin, Adderall & Concerta that are useful in burning off the "brain fog".


Emotional

Emotional symptoms are also common and are thought to be both the normal response to having a debilitating disease and the result of damage to specific areas of the central nervous system that generate and control emotions.

Clinical depression is the most common neuropsychiatric condition: lifetime depression prevalence rates of 40-50% and 12 month prevalence rates around 20% have been typically reported for samples of people with MS; these figures are considerably higher than those for the general population or for people with other chronic illnesses. Many brain-imaging studies have tried to relate depression to lesions in different brain regions with variable success. On balance the evidence seems to favour an association with neuropathology in the left anterior temporal/parietal regions.

Other feelings such as anger, anxiety, frustration, and hopelessness also appear frequently, and suicide is a very real threat since 15% of deaths in MS sufferers are due to this cause.


Fatigue

Fatigue is very common and disabling in MS. At the same time it has a close relationship with depressive symptomatology. When depression is reduced fatigue also tends to improve, so patients should be evaluated for depression before other therapeutic approaches are used. In a similar way other factors like disturbed sleep, chronic pain, poor nutrition, or even some medications can contribute to fatigue; and therefore medical professionals are encouraged to identify and modify them. There are also different medications used to treat fatigue; such as amantadine, or pemoline; as well as psychological interventions of energy conservation; but the effects of all of them are small. For this reason fatigue is a very difficult symptom to manage.


Internuclear ophthalmoplegia

Schematic demonstrating right internuclear ophthalmoplegia, caused by injury of the right medial longitudinal fasciculus

Internuclear ophthalmoplegia is a disorder of conjugate lateral gaze. The affected eye shows impairment of adduction. The partner eye diverges from the affected eye during abduction, producing diplopia; during extreme abduction, compensatory nystagmus can be seen in the partner eye. Diplopia means double vision while nystagmus is involuntary eye movement characterized by alternating smooth pursuit in one direction and a saccadic movement in the other direction.

Internuclear ophthalmoplegia occurs when MS affects a part of the brain stem called the medial longitudinal fasciculus, which is responsible for communication between the two eyes by connecting the abducens nucleus of one side to the oculomotor nucleus of the opposite side. This results in the failure of the medial rectus muscle to contract appropriately, so that the eyes do not move equally (called disconjugate gaze).

Different drugs as well as optic compensatory systems and prisms can be used to improve this symptoms. Surgery can also be used in some cases for this problem.


Mobility restrictions

Restrictions in mobility (walking, transfers, bed mobility) are common in individuals suffering from multiple sclerosis. Within 10 years after the onset of MS one-third of patients reach a score of 6 on the Expanded Disability Status Scale (requiring the use of a unilateral walking aid),and by 30 years the proportion increases to 83%. Within 5 years the Expanded Disability Status Score is 6 in 50% of those with the progressive form of MS.

In MS a wide range of impairments may exist which can act either alone or in combination to impact directly on a person's balance, function and mobility. Such impairments include fatigue, weakness, hypertonicity, low exercise tolerance, impaired balance, ataxia and tremor.

Interventions may be aimed at the level of the impairments that reduce mobility; or at the level of disability. At this second level interventions include provision, education and instruction in use of equipment such as walking aids, wheelchairs, motorized scooters and car adaptations; and instruction about compensatory strategies to accomplish an activity, (for example,undertaking safe transfers by pivoting in a flexed posture rather than standing up and stepping around)


Optic neuritis

Up to 50% of patients with MS will develop an episode of optic neuritis, and 20% of the time optic neuritis is the presenting sign of MS. The presence of demyelinating white matter lesions on brain MRI at the time of presentation of optic neuritis is the strongest predictor for developing clinically definite MS. Almost half of the patients with optic neuritis have white matter lesions consistent with multiple sclerosis. At five years follow-up, the overall risk of developing MS is 30%, with or without MRI lesions. Patients with a normal MRI still develop MS (16%), but at a lower rate compared to those patients with three or more MRI lesions (51%). From the other perspective, however, almost half (44%) of patients with any demyelinating lesions on MRI at presentation will not have developed MS ten years later.

Individuals experience rapid onset of pain in one eye, followed by blurry vision in part or all of the visual field of that eye. Inflammation of the optic nerve causes loss of vision usually because of the swelling and destruction of the myelin sheath covering the optic nerve.

The blurred vision usually resolves within ten weeks, but individuals are often left with less vivid color vision (especially red) in the affected eye.

Systemic intravenous treatment with corticosteroids, which may quicken the healing of the optic nerve, prevent complete loss of vision, and delay the onset of other symptoms, is often recommended.


Pain

Pain is a common symptom in MS; appearing in 55% of patients at some point of their disease process; specially as time passes. It is strong and debilitating and has a profound effect in the quality of life and mental health of the sufferer. It usually appears after a lesion to the ascending or descending tracts that control the transmission of painful stimulus. such as the anterolateral system, but many other causes are also possible. Most frequent pains reported are headaches (40%), dysesthetic limb pain (19%), back pain (17%), and painful spasms (11%).

Acute pain is mainly due to optic neuritis, being corticosteroids the best treatment available; to trigeminal neuralgia, to Lhermitte's sign or to dysesthesias. Subacute pain is usually secondary to the disease and can be consequence of being too much time in the same position, urinary retention, infected skin ulcers and many others. Treatment will depend on cause. Chronic pain is very common and the harder to treat being its most common cause dysesthesias.

Trigeminal neuralgia

Trigeminal neuralgia or "tic douloureux", is a disorder of the trigeminal nerve that causes episodes of intense pain in the eyes, lips, nose, scalp, forehead, and jaw. It affects 1 to 2% of MS patients during their disease. The episodes of pain occur paroxysmally, or suddenly; and the patients describe it as trigger area on the face, so sensitive that touching or even air currents can bring an episode of pain. Usually it's successfully treated with anticonvulsants such as carbamazepine or phenytoin but others such as gabapentin can be used. When drugs are not effective enough surgery may be recommended. Further damage to the nerve to prevent the transmission of pain (Rhyzotomy) has proven its efficacy; however the beneficial effects and risks in multiple sclerosis patients of those procedures that consist in relieving the pressure on the nerve are still under discussion.

Lhermitte's sign

Lhermitte's sign is an electrical sensation that runs down the back and into the limbs, and is produced by bending the neck forward. The sign suggests a lesion of the dorsal columns of the cervical cord or of the caudal medulla; correlating significantly with cervical MRI abnormalities. Between 25 and 40% of MS patients report having Lhermitte's sign during the course of their illness.

Dysesthesias

Dysesthesias are disagreeable sensations produced by ordinary stimuli. The abnormal sensations are often described as painful feelings such as burning, wetness, itching, electric shock or pins and needles; and are caused by lesions of the peripheral or central sensory pathways. Both Lhermitte's sign and painful dysesthesias usually respond well to treatment with carbamazepine, clonazepam or amitriptyline.


Sexual

Sexual dysfunction (SD) is one of many symptoms affecting persons with a diagnosis of multiple sclerosis (MS) and other neurological disease. SD in men encompasses both erectile and ejaculatory disorder. The prevalence of SD in men with MS ranges from 75 to 91% (O'Leary et al., 2007). Erectile dysfunction appears to be the most common form of SD documented in MS. SD may be due to alteration of the ejaculatory reflexe which may be affected by neurological conditions such as MS.


Spasticity

Spasticity is characterised by increased stiffness and slowness in limb movement, the development of certain postures, an association with weakness of voluntary muscle power, and with involuntary and sometimes painful spasms of limbs. A physiotherapist can help to reduce spasticity and avoid the development of contractures with techniques such as passive stretching. There is evidence, albeit limited, of the clinical effectiveness of baclofen, dantrolene, diazepam, and tizanidin. In the most complicated cases intrathecal injections of baclofen can be used. There are also palliative measures like castings, splints or customised seatings.


Transverse myelitis

Some MS patients develop rapid onset of numbness, weakness, bowel or bladder dysfunction, and/or loss of muscle function, typically in the lower half of the body. This is the result of MS attacking the spinal cord. The symptoms and signs depend upon the level of the spinal cord involved and the extent of the involvement.

Prognosis for complete recovery is generally poor. Recovery from transverse myelitis usually begins between weeks 2 and 12 following onset and may continue for up to 2 years in some patients and as many as 80% of individuals with transverse myelitis are left with lasting disabilities.

Treatment is usually symptomatic only, corticosteroids being used with limited success.


Tremor and ataxia

Tremor is an unintentional, somewhat rhythmic, muscle movement involving to-and-fro movements (oscillations) of one or more parts of the body. It is the most common of all involuntary movements and can affect the hands, arms, head, face, vocal cords, trunk, and legs. Ataxia is an unsteady and clumsy motion of the limbs or torso due to a failure of the gross coordination of muscle movements. People with ataxia experience a failure of muscle control in their arms and legs, resulting in a lack of balance and coordination or a disturbance of gait.

Tremor and ataxia are frequent in MS. They present in 25 to 60% of patients. They can be very disabling and embarrassing, and are difficult to manage. The origin of tremor in MS is difficult to precise but it can be due to a mixture of different factors such as damage to the cerebellar connections, weakness, spasticity, etc.

In the treatment of tremor many medications have been proposed; however their efficacy is very limited. Medications that have been reported to provide some relief are isoniazid, carbamazepine, propranolol; and gluthetimide, but published evidence of effectiveness is very limited. Physical therapy is not indicated as a treatment for tremor or ataxia; however, the use of different orthese devices can help. An example is the use of wrist bandages with weights, which can be useful to increase the inertia of movement and therefore reduce tremor. Daily use objects have also to be adapted so they are easier to grab and use.

If all these measures fail some patients are candidates for thalamus surgery. This kind of surgery can be both a thalamotomy or the implantation of a thalamic stimulator. Complications are frequent (30% in thalamotomy and 10% in deep brain stimulation) and include a worsening of ataxia, dysarthria and hemiparesis. Thalamotomy is a more efficacious surgical treatment for intractable MS tremor, however the higher incidence of persistent neurological deficits in patients receiving lesional surgery supports the use of deep brain stimulation as the preferred surgical strategy.



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