INTRODUCTION
A lot of people feel confused about which diseases are covered under the umbrella term Chronic Neurological Conditions. India’s RPWD Act 2016 mentions Chronic Neurological Conditions as one of the recognized disabilities. In order to provide a meaning or definition of these conditions, the guidelines give examples of Multiple Sclerosis and Parkinson’s disease.
Definition and Meaning of Chronic Neurological Conditions
The 4th July 2018 notification of the Ministry of Social Justice and Empowerment give definition as below:
Chronic
neurological conditions, such as—
(i) “multiple sclerosis” means an inflammatory, nervous system disease in which
the myelin sheaths around the axons of nerve cells of the brain and spinal cord
are damaged, leading to demyelination and affecting the ability of nerve cells
in the brain and spinal cord to communicate with each other;
(ii) “Parkinson’s disease” means a progressive disease of the nervous system
marked by tremor, muscular rigidity, and slow, imprecise movement, chiefly
affecting middle-aged and elderly people associated with degeneration of
the basal ganglia of the brain and a deficiency of the neurotransmitter
dopamine.
But MS and PD have been cited only as examples.
Neurological disorders are diseases of the central and peripheral nervous system. In other words, the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscles. These disorders include epilepsy, Alzheimer disease and other dementias, cerebrovascular diseases including stroke, migraine and other headache disorders, multiple sclerosis, Parkinson's disease, neuroinfections, brain tumours, traumatic disorders of the nervous system due to head trauma, and neurological disorders as a result of malnutrition.
Many bacterial (i.e. Mycobacterial tuberculosis, Neisseria meningitides), viral (i.e. Human Immunodeficiency Virus (HIV), Enteroviruses, West Nile Virus, Zika), fungal (i.e. Cryptococcus, Aspergillus), and parasitic (i.e. malaria, Chagas) infections can affect the nervous system. Neurological symptoms may occur due to the infection itself, or due to an immune response.
TYPES OF CHRONIC NEUROLOGICAL CONDITIONS
There are other conditions that can be categorized under Chronic Neurological Conditions. Some more examples can be:
1. Alzheimer’s disease and Dementia
2. Parkinson’s disease
3. Dystonia
4. ALS (Lou Gehrig’s disease)
5. Huntington’s disease
6. Neuromuscular disease
7. Multiple sclerosis
8. Epilepsy
9. Stroke
ALZHEIMER’S DISEASE AND OTHER DEMENTIAS
Dementia is a deterioration of intellectual function and other cognitive skills that is of sufficient severity to interfere with social or occupational functioning. Of the many diseases that lead to dementia, AD is the most common cause worldwide among people age 65 and older, followed by vascular dementia, mixed dementia consisting of AD plus vascular dementia, and dementia caused by general medical conditions. Although distinguishing AD from other causes of dementia is important, particularly for treatment with acetylcholinesterase inhibitors, the burden from all causes of dementia is similar. Although the discussion in this chapter deals mostly with AD, the role of treatable dementias in developing countries is important as it can reduce the burden of caring in families. In the early stages of Alzheimer's disease, memory loss is mild, but in its late-stage, individuals lose the ability to complete daily tasks, carry on a conversation, and respond to their environment. Alzheimer's disease is the sixth leading cause of death in the United States. Those with Alzheimer's disease live an average of eight years after their symptoms become noticeable. However, depending on age and other health conditions, survival can range from four to 20 years.
Risk and Protective Factors and Survivorship
Three separate genes (APP, PS1, and PS2) are linked to earlyonset, familial AD. Another gene (APO E4) is a risk factor for late-onset, nonfamilial cases (Henderson and Jorm 2000). Other genes have been implicated but not confirmed in large studies. Other risk factors reported in the literature include increasing age, positive family history of dementia, female gender (but this factor is controversial), lower level of education, several medical conditions, and exposure to such environmental factors as organic solvents and aluminum. Protective factors reported in the literature include a higher level of education, a specific gene (APO E2), the intake of antioxidants, and the use of some anti-inflammatory medications. The use of estrogen supplements for women was believed to be a protective factor for AD (Henderson 1997), but a recent study of women taking a combination of estrogen and progesterone showed that these women had twice the risk of developing dementia than women taking a placebo. Studies from developed countries have reported median survival after the onset of dementia symptoms ranging from 5.0 years to 9.3 years (Walsh, Welch, and Larson 1990). In developing countries, the reported median survival was 3.3 years for all demented subjects and 2.7 years for those with AD.
PARKINSON’S DISEASE
PD is characterized by bradykinesia, resting tremor, cogwheel rigidity, postural reflex impairment, progressive course, and good response to dopaminergic therapy. Other distinct forms of parkinsonism include relatively rare genetic forms and the less common neurodegenerations with multiple system involvement or significant striatal lesions (for example, progressive supranuclear palsy or multiple system atrophy). Parkinsonism secondary to external causes, such as manganese poisoning or carbon monoxide poisoning, although now rare, is referred to as secondary parkinsonism. Because the burden of these diseases to the patient is similar to or greater than that for PD and there is no evidence for addressing these disorders separately, they will not be distinguished here.
Parkinson's signs and symptoms may include:
· Tremor. A tremor, or shaking, usually begins in a limb, often your hand or fingers. You may a rub your thumb and forefinger back-and-forth, known as a pill-rolling tremor. Your hand may tremor when it's at rest.
· Slowed movement (bradykinesia). Over time, Parkinson's disease may slow your movement, making simple tasks difficult and time-consuming. Your steps may become shorter when you walk. It may be difficult to get out of a chair. You may drag your feet as you try to walk.
· Rigid muscles. Muscle stiffness may occur in any part of your body. The stiff muscles can be painful and limit your range of motion.
· Impaired posture and balance. Your posture may become stooped, or you may have balance problems as a result of Parkinson's disease.
· Loss of automatic movements. You may have a decreased ability to perform unconscious movements, including blinking, smiling or swinging your arms when you walk.
· Speech changes. You may speak softly, quickly, slur or hesitate before talking. Your speech may be more of a monotone rather than with the usual inflections.
· Writing changes. It may become hard to write, and your writing may appear small.
Causes and Risk Factors
The cause of PD is unknown. A specific environmental risk factor has not been identified. Pure genetic forms account for 10 to 15 percent of cases or fewer. Increasing age and male gender are risk factors worldwide. Exposure to toxins, head trauma, frequent infections, diets high in animal fat, and midlife adiposity have been reported to increase PD risk, but none do so consistently. The most consistent association is an inverse association with cigarette smoking and caffeine consumption, suggesting a protective effect.
EPILEPSY
Epilepsy is a common brain disorder characterized by two or more unprovoked seizures. Seizures are discrete events caused by transient, hypersynchronous, abnormal neuronal activity. Seizures may occur in close temporal association with a variety of acute medical and neurological diseases, such as acute stroke, sepsis, or alcohol withdrawal. However, the vast majority of seizures have no immediate identifiable cause. Epilepsy can be broadly divided into three categories: idiopathic epilepsy (for example primary generalized childhood-onset absence epilepsy), which is thought to have a genetic basis; secondary or symptomatic epilepsy, which is caused by a known central nervous system injury or disorder, such as infection, stroke, traumatic brain injury, or cerebral dysgenesis; and cryptogenic epilepsy, for which there is no clear evidence of an etiological factor. Idiopathic and cryptogenic cases represent approximately 70 percent of epilepsy cases; the remaining 30 percent are symptomatic (secondary). Seizures are the main symptom of epilepsy. Symptoms differ from person to person and according to the type of seizure.
When seizures appear to result from abnormal activity in just one area of your brain, they're called focal (partial) seizures. These seizures fall into two categories:
· Focal seizures without loss of consciousness. Once called simple partial seizures, these seizures don't cause a loss of consciousness. They may alter emotions or change the way things look, smell, feel, taste or sound. They may also result in involuntary jerking of a body part, such as an arm or leg, and spontaneous sensory symptoms such as tingling, dizziness and flashing lights.
· Focal seizures with impaired awareness. Once called complex partial seizures, these seizures involve a change or loss of consciousness or awareness. During a complex partial seizure, you may stare into space and not respond normally to your environment or perform repetitive movements, such as hand rubbing, chewing, swallowing or walking in circles.
Symptoms of focal seizures may be confused with other neurological disorders, such as migraine, narcolepsy or mental illness. A thorough examination and testing are needed to distinguish epilepsy from other disorders.
Seizures that appear to involve all areas of the brain are called generalized seizures. Six types of generalized seizures exist.
· Absence seizures. Absence seizures, previously known as petit mal seizures, often occur in children and are characterized by staring into space or subtle body movements such as eye blinking or lip smacking. These seizures may occur in clusters and cause a brief loss of awareness.
· Tonic seizures. Tonic seizures cause stiffening of your muscles. These seizures usually affect muscles in your back, arms and legs and may cause you to fall to the ground.
· Atonic seizures. Atonic seizures, also known as drop seizures, cause a loss of muscle control, which may cause you to suddenly collapse or fall down.
· Clonic seizures. Clonic seizures are associated with repeated or rhythmic, jerking muscle movements. These seizures usually affect the neck, face and arms.
· Myoclonic seizures. Myoclonic seizures usually appear as sudden brief jerks or twitches of your arms and legs.
· Tonic-clonic seizures. Tonic-clonic seizures, previously known as grand mal seizures, are the most dramatic type of epileptic seizure and can cause an abrupt loss of consciousness, body stiffening and shaking, and sometimes loss of bladder control or biting your tongue.
Risk Factors
A reported risk factor for idiopathic (presumed genetic) epilepsy is family history of epilepsy. Reported risk factors for symptomatic epilepsy include prenatal or perinatal causes (obstetric complications, prematurity, low birthweight, neonatal asphyxia). Recent data suggest that the effect of obstetric complications or neonatal asphyxia may have been overemphasized. Prematurity, low birthweight, and neonatal seizures may be independent risk factors as well as markers of underlying disease. Other causes include traumatic brain injuries, central nervous system infections, cerebrovascular disease, brain tumors, and neurodegenerative diseases. Developmental disabilities are not a risk factor for epilepsy in themselves, but they may be associated with seizure disorder.
STROKE
Stroke, also known as cerebrovascular accident or brain attack, is a syndrome caused by an interruption in the flow of blood to part of the brain caused either by occlusion of a blood vessel (ischemic stroke) or rupture of a blood vessel (hemorrhagic stroke). The interruption in blood flow deprives the brain of nutrients and oxygen, resulting in injury to cells in the affected vascular territory of the brain. The occlusion of a blood vessel can sometimes be temporary and present as a reversible neurological deficit, which is termed a transient ischemic attack. Even though stroke is a clinical diagnosis, brain imaging is required to distinguish ischemic stroke from hemorrhagic stroke. When imaging is unavailable, clinical scores can be useful to identify patients with intracerebral hemorrhage. Stroke symptoms can include:
Types of stroke
Strokes fall into three main categories: transient ischemic attack (TIA), ischemic stroke, and hemorrhagic stroke. These categories are further broken down into other types of strokes, including:
ü embolic stroke
ü thrombotic stroke
ü intracerebral stroke
ü subarachnoid stroke
· Ischemic stroke
During an ischemic stroke, the arteries supplying blood to the brain narrow or become blocked. These blockages are caused by blood clots or blood flow that’s severely reduced. They can also be caused by pieces of plaque due to atherosclerosis breaking off and blocking a blood vessel. The two most common types of ischemic strokes are thrombotic and embolic. A thrombotic stroke happens when a blood clot forms in one of the arteries supplying blood to the brain. The clot passes through the bloodstream and becomes lodged, which blocks blood flow. An embolic stroke is when a blood clot or other debris forms in another part of the body and then travels to the brain.
· Embolic stroke
An embolic stroke is one of two types of ischemic strokes. It occurs when a blood clot forms in another part of the body — often the heart or arteries in the upper chest and neck — and moves through the bloodstream to the brain. The clot gets stuck in the brain’s arteries, where it stops the flow of blood and causes a stroke. An embolic stroke may be the result of a heart condition. Atrial fibrillation, a common type of irregular heartbeat, can cause blood clots to develop in the heart. These clots may dislodge and travel through the bloodstream and into the brain.
· Transient ischemic attack (TIA)
A transient ischemic attack, often called a TIA or ministroke, occurs when blood flow to the brain is blocked temporarily. Symptoms, which are similar to those of a full stroke, are typically temporary and disappear after a few minutes or hours. A TIA is usually caused by a blood clot. It serves as a warning of a future stroke, so don’t ignore a TIA. According to the CDC, more than one-third of people who experience a TIA and don’t get treatment have a major stroke within a year. Up to 10 to 15 percent of people who experience a TIA have a major stroke within three months.
Risk Factors
Risk factors for stroke in general are similar to those for cardiovascular disease. Moreover, risk factors for first stroke and recurrence of stroke are also similar if they remain uncontrolled after the first attack (see chapter 33). Increasing age, particularly after 55, is one of the most important risk factors for stroke. Although stroke is more prevalent among men, strokerelated fatality rates are higher among women. Hypertension is the most important modifiable determinant of both first and recurrent stroke. The association between blood pressure and stroke in East Asian populations seems stronger than in Western populations. Other risk factors include smoking, environmental exposure to tobacco smoke, dyslipidemia, atrial fibrillation, diabetes and impaired glucose tolerance, generalized and abdominal obesity, physical inactivity, excess alcohol consumption, increased homocysteine levels, drug abuse, hemostatic factors, and existing cerebrovascular disease. In developing countries, rheumatic heart disease leading to embolic stroke is also a major cause. This risk factor is declining in importance with the control of rheumatic fever. Dehydration in postpartum women can lead to a stroke, particularly in remote areas where deliveries are conducted at home.
MULTIPLE SECLEROSIS
Multiple sclerosis (MS) is an inflammatory demyelinating condition of the central nervous system (CNS) that is generally considered to be autoimmune in nature. In people with MS, the immune trigger is unknown, but the targets are myelinated CNS tracts. In regions of inflammation, breakdown of the blood–brain barrier occurs and destruction of myelin ensues, with axonal damage, gliosis and the formation of sclerotic plaques. Plaques (MS lesions) may form in the CNS white matter in any location (and also in grey matter); thus, clinical presentations may be diverse. Continuing lesion formation in MS often leads to physical disability and, not infrequently, to cognitive decline. Multiple sclerosis signs and symptoms may differ greatly from person to person and over the course of the disease depending on the location of affected nerve fibers. Symptoms often affect movement, such as:
· Numbness or weakness in one or more limbs that typically occurs on one side of your body at a time, or your legs and trunk
· Electric-shock sensations that occur with certain neck movements, especially bending the neck forward (Lhermitte sign)
· Tremor, lack of coordination or unsteady gait
Vision problems are also common, including:
· Partial or complete loss of vision, usually in one eye at a time, often with pain during eye movement
· Prolonged double vision
· Blurry vision
Multiple sclerosis symptoms may also include:
· Slurred speech
· Fatigue
· Dizziness
· Tingling or pain in parts of your body
· Problems with sexual, bowel and bladder function
Types of MS
There are four types of MS:
Clinically isolated syndrome (CIS): This is a single, first episode, with symptoms lasting at least 24 hours. If another episode occurs at a later date, a doctor will diagnose relapse-remitting MS.
Relapse-remitting MS (RRMS): This is the most common form, affecting around 85% of people with MS. RRMS involves episodes of new or increasing symptoms, followed by periods of remission, during which symptoms go away partially or totally.
Primary progressive MS (PPMS): Symptoms worsen progressively, without early relapses or remissions. Some people may experience times of stability and periods when symptoms worsen and then get better. Around 15% of people with MS have PPMS.
Secondary progressive MS (SPMS): At first, people will experience episodes of relapse and remission, but then the disease will start to progress steadily.
Risk Factors
The worldwide distribution of MS can be only an indirect refl ection of its cause, implicating some environmental factor that varies with latitude, and can be interpreted in at least three different ways in the search for clues to a specifi c etiology. First, an environmental risk factor may be more common in temperate than tropical climates. Second, such a factor may be more common in tropical climates, where it is acquired at an earlier age and consequently has less impact. Third, this factor may be equally common in all regions, but the chance of its acquisition or of the manifestation of symptoms is either increased by some enhancing factor present in temperate climates or reduced by a protective factor present in tropical areas. Among those factors that have been most closely scrutinized are:
· infections, including a number of viral infections such as measles and Epstein–Barr virus;
· climate and solar conditions;
· living conditions;
· diet and trace elements.
It is now generally accepted that the etiology of MS involves some interplay of genetic and environmental factors. Evidence of racial or ethnic resistance, the increased risk among MS family members, and elevated monozygotic twin concordance rate all favour a genetic contribution to acquisition of the disease. The studies from which this evidence is derived, however, also indicate that heredity cannot entirely explain the occurrence of MS. This is underlined by the fact that no population-based study of monozygotic twins has found a concordance rate in excess of 30%. Some environmental factor, such as a virus or toxin, must still play a role.
Dystonia is a very complex, highly variable neurological movement disorder characterized by involuntary muscle contractions. Dystonia results from abnormal functioning of the basal ganglia, a deep part of the brain which helps control coordination of movement. These regions of the brain control the speed and fluidity of movement and prevent unwanted movements. Patients with dystonia may experience uncontrollable twisting, repetitive movements or abnormal postures and positions. These can affect any part of the body, including the arms, legs, trunk, face and vocal cords.
Depending on the part of the body affected, dystonia can seriously impact daily functions. For example, if neck muscles are affected, a patient may have difficulty chewing and swallowing. Though not life-threatening, the involuntary nature of the disorder may be embarrassing, causing emotional distress or depression in some individuals. There are a number of local support groups throughout the United States that can help address some of these issues, but patients may need to be treated separately for mental health issues caused by the challenges of coping with this disorder.
· Childhood onset – 0 to age 12
· Adolescent onset – age 13 to 20
· Adult onset – older than age 20
Focal dystonia is limited to one area of the body and can affect the neck (cervical dystonia or spasmodic torticollis), eyes (blepharospasm), jaw/mouth/lower face (oromandibular dystonia), vocal cords (laryngeal dystonia) or arms/legs (limb dystonia). Other less common types of focal dystonias can cause unusual stretching, bending or twisting of the trunk (truncal dystonia) or sustained contractions and involuntary, writhing movements of the abdominal wall (abdominal wall dystonia).
Focal dystonia more commonly affects people in their 40s and 50s and is frequently referred to as adult-onset dystonia. Women are affected about three times more frequently than men. In general, focal dystonias are classified as primary (idiopathic) and are not hereditary.
Segmental dystonia affects two or more parts of the body that are adjacent or close to one another. Up to 30 percent of people with focal dystonia have spasms in areas adjacent to the primary site. A common form of segmental dystonia affects the eyelids, jaw, mouth and lower face.
Other types of dystonia include multifocal, which involves two or more body parts distant from one another; hemidystonia, which affects half of the body; and generalized, which begins with leg involvement, but generally spreads to one or more additional regions of the body.
Primary (idiopathic) dystonia is the only sign, and secondary causes have been ruled out. Most primary dystonias are variable, have adult onse, and are focal or segmental in nature. However, there are specific primary dystonias with childhood or adolescent onset that have been linked to genetic mutations.
The majority of early-onset primary dystonias, which may appear during childhood or early adulthood, are due to mutations of a gene known as DYT1. This gene has been mapped to the long arm of chromosome 9 at 9q34.1. In about 90 to 95 percent of cases, symptoms begin in a limb and then spread to other regions of the body. This form of dystonia has an average age of onset of 12 and seldom develops after age 29.
DYT6 dystonia is an autosomal dominant primary dystonia that has been mapped to chromosome 8 (8p21q22). It is rarer than DYT1 dystonia and has been studied in two Mennonite families in the United States. In nearly all individuals with this form of dystonia, the disorder begins at an initial site but spreads to multiple body regions, most commonly the limbs, head or neck. Severe difficulties with speech articulation have been noted.
Other familial primary dystonias identified are DYT7, DYT2, and DYT4, all of which have been noted in specific ethnic groups, primarily of European descent.
Secondary (symptomatic) results primarily from secondary causes. These include environmental, such as exposure to carbon monoxide, cyanide, manganese or methanol; underlying conditions and diseases such as brain tumors, cerebral palsy, Parkinson’s disease, stroke, multiple sclerosis, hypoparathyroidism or vascular malformations; brain/spinal cord injuries; inflammatory, infectious or postinfectious brain conditions; and specific medications.
Dystonia-plus syndromes results from nondegenerative, neurochemical disorders associated with other neurological conditions. Dystonia-plus syndromes include dopa-responsive dystonia (DRD) or Segawa syndrome, rapid-onset dystonia-parkinsonism (RDP) and myoclonus-dystonia.
Heredodegenerative dystonia generally results from neurodegenerative disorders in which other neurological symptoms are present and in which heredity plays a role. These include numerous disorders such as certain X-linked recessive, autosomal dominant, autosomal recessive and/or parkinsonian syndromes. Included in this category: X-linked dystonia-parkinsonism (Lubag), Huntington's disease, Wilson's disease, neuroacanthocytosis, Rett’s syndrome, Parkinson's disease and juvenile parkinsonism.
ALS (LOU GEHRIG’S DISEASE)
ALS, or amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord. A-myo-trophic comes from the Greek language. "A" means no. "Myo" refers to muscle, and "Trophic" means nourishment – "No muscle nourishment." When a muscle has no nourishment, it "atrophies" or wastes away. "Lateral" identifies the areas in a person's spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates, it leads to scarring or hardening ("sclerosis") in the region.
Motor neurons reach from the brain to the spinal cord and from the spinal cord to the muscles throughout the body. The progressive degeneration of the motor neurons in ALS eventually leads to their demise. When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost. With voluntary muscle action progressively affected, people may lose the ability to speak, eat, move and breathe. The motor nerves that are affected when you have ALS are the motor neurons that provide voluntary movements and muscle control. Examples of voluntary movements are making the effort to reach for a smart phone or step off a curb. These actions are controlled by the muscles in the arms and legs. Signs and symptoms might include:
· Difficulty walking or doing normal daily activities
· Tripping and falling
· Weakness in your leg, feet or ankles
· Hand weakness or clumsiness
· Slurred speech or trouble swallowing
· Muscle cramps and twitching in your arms, shoulders and tongue
· Inappropriate crying, laughing or yawning
· Cognitive and behavioral changes
HUNTINGTON’S DISEASE
Huntington’s disease (HD) is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities usually during their prime working years and has no cure. HD is known as the quintessential family disease because every child of a parent with HD has a 50/50 chance of inheriting the faulty gene. The symptoms of HD are described as having ALS, Parkinson’s and Alzheimer’s – simultaneously.
Symptoms usually appear between the ages of 30 to 50, and worsen over a 10 to 25-year period. Ultimately, the weakened individual succumbs to pneumonia, heart failure or other complications. Everyone has the gene that causes HD, but only those that inherit the expansion of the gene will develop HD and perhaps pass it on to each of their children. Every person who inherits the expanded HD gene will eventually develop the disease. Over time, HD affects the individual’s ability to reason, walk and speak.
· Personality changes, mood swings & depression
· Forgetfulness & impaired judgment
· Unsteady gait & involuntary movements (chorea)
· Slurred speech, difficulty in swallowing & significant weight loss
Causes
Huntington's disease is caused by an inherited defect in a single gene. Huntington's disease is an autosomal dominant disorder, which means that a person needs only one copy of the defective gene to develop the disorder.
With the exception of genes on the sex chromosomes, a person inherits two copies of every gene — one copy from each parent. A parent with a defective gene could pass along the defective copy of the gene or the healthy copy. Each child in the family, therefore, has a 50% chance of inheriting the gene that causes the genetic disorder.
NEUROMUSCULAR DISEASE
Neuromuscular disease is a broad term that encompasses many diseases and ailments that impair the functioning of the muscles. These may directly involve the muscle directly or indirectly by involving the nerves or the Neuromuscular junction (the meeting point of the motor nerves and muscle fibre). The symptoms of Neuromuscular diseases may include- Numbness, painful abnormal sensation, muscle weakness, muscle atrophy, muscle pain or twitching (fasciculation). The Neuromuscular diseases may be categorised into the following groups:
· Diseases of Anterior Horn Cells (Junction between spinal cord and motor nerve) – Motor Neurone Diseases
· Diseases involving motor and sensory nerves – Peripheral Neuropathies
· Disorders of the Neuromuscular function – Myasthenia Gravis and related diseases
· Diseases of the muscles (Myopathies)- Muscular Dystrophy and Inflammatory myopathies
Motor neurone diseases
These are characterised by progressive degeneration and loss of motor neurons in the spinal cord, with or without similar lesions in motor nuclei of the brainstem. The cells lost are replaced by gliosis (scar tissue). The term Motor Neurone Diseases (singular) is however reserved for the adult disease - Amyotrophic Lateral Sclerosis (ALS) in which both upper and lower motor neurons are affected. In ALS, the weakness may commence in the leg, hands, proximal arm or oropharynx (slurred speech or difficulty in swallowing). Often the hands are affected first, mostly asymmetrically. Painless difficulties with buttoning or turning of a key are ominous symptoms in midlife. Fasciculation may be seen in the tongue and proximal muscles of upper limb.
Peripheral neuropathies
This is a term used to describe syndrome resulting from diffuse lesions of the peripheral nerves, usually manifested by weakness, sensory loss, pain and autonomic dysfunction. ‘Neuritis’ is a term typically reserved for inflammatory disorder of the nerves, resulting from infection or autoimmunity. Symptoms of polyneuropathy include acral pain, paraesthesia, weakness and sensory loss. The pain may be spontaneous or elicited by stimulation of the skin and may be sharp or burning. Paraesthesias are usually describes as numbness, tingling, buzzing, burning or a feeling of constriction. In these patients a detailed family, social, medical and drug history in addition to thorough examination, followed by NCV / EMG and other lab tests, is needed to clinch the diagnosis. Some of the common causes in India include- Immune mediated (Guillain Barre Syndrome), B12 deficiency, Diabetes, Drug related, toxins and Renal failure.
Disorders of neuromuscular junction
Muscle diseases caused by a defect of Nerve Signal transmission owing to antibody medicated attack or acetyl- choline receptors at Neuromuscular junction. The abnormal antibodies are produced in the thymus gland and bone marrow. The characteristic fluctuating nature of the myasthenic weakness is unlike any other disease.
The weakness varies in the course of a single day, sometimes within minutes and it varies from day to day or over longer periods. It is usually worse towards the evening. The abnormal antibodies can be detected in the serum of around 85% of patients with generalised Myasthenia. Sometimes the condition can be localised to the eyes and is called Ocular Myasthenia. Drugs, Plasmaphareses, Intravenous Immunoglobulin, removal of thymus and steroids are various modes of treatment.
Progressive muscular dystrophies
These are primary, inherited, progressive degenerative disorders of muscle. The limbs-girdle, facial and sometimes the heart muscles may be affected and become weak. The disease shows slow, relentless progression, without any pain or sensory loss. The condition is recognised as heritable, even though there is no other case in the family. There is no curative therapy and gene therapy has not yet been successful, although for one of the common forms seen in childhood i.e. Duchenne Muscular Dystrophy, a disease modifying treatment is available. The disease characteristically involves muscles in one group in a selective manner. Diagnosis is by EMG and muscle biopsy.
THERAPIES FOR NEUROLOGICAL DISORDERS
Apart from the issue of a cure, sometimes patients with neurological issues can be placed in rehabilitation as part of an effort to restore some lost function. This is usually a hopeful sign, as it’s rare to find a patient assigned to therapy when there’s little to no hope of at least a partial recovery. Therapies for neurological disorders may often consist of:
· Lifestyle changes to either prevent or minimize the impact of such conditions
· Physiotherapy to manage the symptoms and restore some function
· Pain management, as many impairments can be associated with considerable discomfort
· Medication to either restore function or prevent a worsening of the patient’s condition
Cognitive Therapy Treatments
One approach to treating primarily behavioral neurological issues goes by the name of cognitive behavioral therapy, formerly known as talk therapy. CBT focuses on reorienting a patient’s thoughts and behavior related to their disability. While this is obviously not an appropriate response to many disorders of the brain and nervous system, such as Parkinson’s disease or epilepsy, it has shown considerable effect in the treatment of ADHD, anxiety and other mood disorders, and a range of primarily psychogenic impairments. It enjoys several advantages, not the least of which is that it doesn’t present the risk of side effects the way drugs or other interventions might. CBT can often be administered by someone other than a doctor, though it should be administered by licensed therapists. It will frequently be among the first choices for patients, given its generally noninvasive nature.
Other Therapeutic Methods
Clearly, CBT is not called for in the case of patients recovering from a stroke, traumatic injury or degenerative brain diseases. In cases such as these, other therapeutic methods are preferred. These may range from medications such as the neuroleptics (haloperidol and chlorpromazine, for example) used to treat organic disorders of the brain such as schizophrenia, to comparatively simple analgesics, such as ibuprofen, acetaminophen and opiates to treat the painful effects of many neurological ailments.
Residential Inpatient Neurological Treatment Centers
One of the options you may have nearby might be any number of residential inpatient neurological treatment centers. These institutions, sometimes referred to simply as rehab centers, usually focus their attention on treatable disorders, chief among these being alcohol abuse issues and other chemical dependencies.
In addition, rehab centers are often used to treat those with neurological issues, helping patients to gain skilled needed to live balanced, healthy lives.