History

Fact Explanation
Introduction to the disease Motor neuron disease (MND) or amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of unknown etiology. There are forms of motor neuron disease that affect selectively either upper motor neurons( pure upper motor neuron syndrome/primary lateral sclerosis) or lower motor neurons.(Pure lower motor neuron syndrome/progressive muscular atrophy). Combination of both is named as ALS. In USA the term ALS is use to describe all the forms of disease and in UK the umbrella term MND is commonly used.[1]. The lower motor neuron involvement in disease can cause LMN manifestations such as fasciculation, atrophy, weakness, and hyporeflexia, Upper motor neurone involvement will cause UMN symptoms and signs such as spasticity, weakness and hyperreflexia where the progressive bulbar involvement causes bulbar symptoms such as dysarthria and dysphagia.[6],[4].
Dysarthria Progressive bulbar palsy may manifest as progressive disturbance of speech lead in to dysarthria .[2]. The mechanism of dysarthria is a flaccid or spastic paresis of the musculature of the face, tongue, lips, palate, pharynx and larynx. At the early stage of ALS patient may experience a mild change in his voice or speech. Those mild changes include harsh or hoarseness of voice,inappropriate pauses in speech, imprecise consonants, nasal quality speech and a decreased range of pitch and loudness.Slurring can be one of the first symptoms of impaired articulation due to tongue involvement in ALS.[4].
Dysphagia A manifestation of bulbar palsy mainly affecting the lower cranial nerve nuclei.[2]. LMN dysfunction causes a weakened tongue.It interferes with the formation of a bolus or a gutter for liquids. It may also leads to weak soft palate, which fails to close the pharyngeal isthmus on swallowing. It may give rise to nasal escape, and weak pharyngeal musculature resulting in ballooning of the pharynx.The combination of above mechanisms ultimately leads to swallowing difficulty in motor neuron disease.[3].
Pooling and drooling of saliva Occurs due to bulbar palsy. This may cause constant irritation to the patient.[5],[3]. Due to increasing loss of tone and strength in the muscles which control lip closure as well as difficulty in swallowing their saliva,may leads to drooling of saliva.Furthermore the inability to keep the lips closed tends to increased breathing through the mouth causing thickening of oral secretions.Hypopharyngeal muscles weakness causes pooling of saliva in the mouth as well as oropharynx.[4].
Emotional lability A manifestation of upper motor neuron involvement in disease causing pseudobulbar palsy. It may leads to emotional liability symptoms such as pathological laughing and crying.[4]. Depression and anxiety are other commonly found symptoms.[5].
Muscle weakness Loss of motor neurons in the spinal cord In MND causes muscle weakness.[4].The earliest symptom is usually asymmetrical weakness of one extremity or, with bulbar onset disease, slurring of speech.[5].
Muscular atrophy Lower motor neuron involvement in disease causes progressive muscular atrophy.[2]. However the oculomotor muscles and sphincter function remains intact.[5].
Stumbling or poor handgrip Weakness is one of the LMN manifestation. Stumbling or poor handgrip is a common manifestation of ALS due to asymmetrical focal weakness of the extremities.[2].Distal limb weakness is often manifest as increasing difficulty turning a key in a lock or car ignition, unscrewing bottle tops, holding a pen, or gripping objects. Involvement of arms as the first manifestation occurs in about 35% of cases.[5].
Muscle cramps Occurs due to LMN involvement.[2].It is one of the common manifestation of disease. It is commonly seen in lower limbs, where both proximal and distal muscles get affected. However unlike muscle cramps, fatigue is not a prominent early symptom.[5].
Respiratory dysfunction Progressive respiratory dysfunction may resulted due to respiratory muscle weakness due to loss of motor neurons in spinal cord.It may also causes decreased voluntary and reflexive coughing as well.[4]
Dyspnea Not a prominent symptom.Occurs due to weakness of ventilatory muscles.[5].
Orthopnoea Occurs due to diaphragmatic weakness.[5].
Constipation Occured due to pelvic muscle weakness , improper diet, spasticity, as well as drugs such as anticholinergics and opioids.[5].
Paraesthesia and numbness, Paraesthesia and numbness are identified sensory symptoms and present in about 10% of affected individuals.[5].
Pain Present in advanced stage of the disease in about 50% of patients.[5].Muscle cramps, stiff joints, spasticity, abdominal colic due to constipation, and skin pressure responsible for the occurrence of pain.[5].
Peripheral oedema All patients with MND may experienced pedal edema in some part of there life.[5].
Frontotemporal dementia Some patients with ALS may develop this. It is a profound alteration in personality and social conduct characterized by loss of volition and insight, social disinhibition, and distractibility, with preservation of memory function.Deficits can be commonly found in attention, planing, problem solving as well as abstraction. They may also present with language disturbances such as progressive aphasia.[2].
Sleep disturbances Depression, anxiety, immobility pain and sleep apnoea found to be contributory factors for sleep disturbances.[5].
Family history of MND Familial ALS inherited in autosomal dominant, autosomal recessive, or X-linked manner. It is said that 10% have an affected family member in case of FALS. FALS can be categorized by mode of inheritance it can be further subcategorized according to the specific gene or chromosomal location.[2].
Prolong exposure to agro chemicals and heavy metals Prolong exposure to mercury, manganese as well as agrochemicals such as fertilizers, insecticides, herbicides considered as factors which contribute to development of ALS.[2].
References
  1. TALBOT K. Motor neurone disease Postgrad Med J [online] 2002 Sep, 78(923):513-519 [viewed 15 September 2014] Available from: doi:10.1136/pmj.78.923.513
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  3. LEIGHTON SE, BURTON MJ, LUND WS, COCHRANE GM. Swallowing in motor neurone disease. J R Soc Med [online] 1994 Dec, 87(12):801-805 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1295008
  4. KüHNLEIN PETER, GDYNIA HANS-JüRGEN, SPERFELD ANNE-DORTE, LINDNER-PFLEGHAR BEATE, LUDOLPH ALBERT CHRISTIAN, PROSIEGEL MARIO, RIECKER AXEL. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol [online] December, 4(7):366-374 [viewed 15 September 2014] Available from: doi:10.1038/ncpneuro0853
  5. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069
  6. HOWARD R S. Management of motor neurone disease. [online] 2002 December, 78(926):736-741 [viewed 17 September 2014] Available from: doi:10.1136/pmj.78.926.736

Examination

Fact Explanation
Flaccid pareses Occurs in bulbar palsy that resulted due to involvement of LMN in MND. It causes degeneration of the lower motor neurons with involvement of the cranial nerve nuclei in the medulla oblongata and pons which innervate the bulbar muscles causing bulbar palsy.[3]
Tongue fasciculations Occurs in MND due to bulbar palsy.[3].
Muscular atrophy Another manifestation of bulbar palsy.[3].
Laryngospasm Laryngospasm is defined clinically as a paroxysmal episode with the sensation that air cannot be moved in and out, accompanied by inspiratory stridor. Rapid forceful contraction of the laryngeal sphincter may lead to this condition. It may resulted as bulbar manifestation due to functional impairment of neurons involved in swallowing and regurgitation and can be seen in late stage of ALS.[3].
Spasticity of the bulbar muscles The upper motor neuron involvement in ALS causes supranuclear symptoms. It is usually call as "pseudobulbar" palsy. Spasticity of the bulbar muscles is a well known characteristic feature of pseudobulbar palsy. Face, soft palate, pharynx, larynx jaw and tongue are commonly get affected.[3].
Brisk jaw jerk Another examination finding of MND due to pseudobulbar palsy.[3]
Hyperreflexia UMN sign of ALS. Hyperreflexia can be demonstrate in segmental regions of muscle atrophy. And usually there is no associated sensory disturbances.[2]
Babinski's sign Babinski's sign positive in about 50% of affected individuals due to involvement of UMN.[4]
Flexor spasms in the legs Affected individuals may rarely presented with flexor spasms in the leg that resulted due to spasticity. [4].
Fasciculations Occurs due lower motor neuron involvement.[2].Cramps and fasciculations may precede weakness and wasting by several months. At the begining fasciculations are obvious but later on with the progression of the muscle weakness, fasciculations become less marked. Fasciculation can occur in the absence of other lower motor neuron signs. [4].
Foot drop At the presentation of ALS limb involvement is more predominant than bulbar involment. Involvement of lower limb may manifest as foot drop. Onset in the lower extremities is most common for familial ALS .[1],[2]. The involvement of lumbosacral region of the spinal cord at the beginning of the disease is seen in about 40% of patients. Those patients start to trip or stumble due to a unilateral foot drop, or may have difficulty rising from a chair if weakness starts proximally.[4].
References
  1. TALBOT K. Motor neurone disease Postgrad Med J [online] 2002 Sep, 78(923):513-519 [viewed 15 September 2014] Available from: doi:10.1136/pmj.78.923.513
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  3. KüHNLEIN PETER, GDYNIA HANS-JüRGEN, SPERFELD ANNE-DORTE, LINDNER-PFLEGHAR BEATE, LUDOLPH ALBERT CHRISTIAN, PROSIEGEL MARIO, RIECKER AXEL. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol [online] December, 4(7):366-374 [viewed 15 September 2014] Available from: doi:10.1038/ncpneuro0853
  4. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069

Differential Diagnoses

Fact Explanation
Spinal muscular atrophy (SMA) It is characterized by progressive muscle weakness resulting from degeneration and loss of the anterior horn cells in the spinal cord and the brain stem nuclei. Onset vary from before birth to adolescence or young adulthood. Common complications include sleep difficulties, pneumonia,Poor weight gain, joint contractions and scoliosis. It is associated with mutations of SMN1. Disease has several sub types. SMA 0 present with prenatal onset and severe joint contractures, facial diplegia, and respiratory failure. SMA 1 onset occurs before age six months where SMA 2 onset between age six and 12 months.SMA III occurs after 1 year SMA IV, is adult onset disease.[1].
Spinal and bulbar muscular atrophy It is a gradually progressive neuromuscular disorder. In there degeneration of lower motor neurons results in muscle weakness, muscle atrophy, and fasciculations. It is an X-linked disorder,occurs only in males. Gynecomastia, testicular atrophy, and reduced fertility as a result of mild androgen insensitivity is commonly found features of affected individuals.[2].
Hereditary spastic paraplegias (HSPs) They are clinically and genetically heterogeneous disorders.Lower extremity spasticity and weakness occurring in variable proportions are characteristic features. Symptoms start at childhood and progress slowly. When symptoms begin in very early childhood, they may be non-progressive and resemble spastic diplegic cerebral palsy.[3].
Hexosaminidase A deficiency They are group of neurodegenerative disorders caused by intralysosomal storage of the specific glycosphingolipid, GM2 ganglioside. The acute infantile variant is prototype hexosaminidase A deficiency commonly named as Tay-Sachs disease. It causes progressive weakness, loss of motor skills and decreased attentiveness. They may also show increased startle response as well as seizures, blindness, spasticity ultimately lead in to death before 4 years. Sub acute, chronic, and adult-onset variants of hexosaminidase A deficiency have later onsets and slower progression. They may develop spinocerebellar degeneration, motor neuron disease as well as dystonia.[4].
Multiple sclerosis (MS) It is an inflammatory, demyelinating, neurodegenerative disorder of the central nervous system of unknown etiology. Peak age of onset is 20-40 years. Women commonly affect than men.Sensory disturbance of the limbs, partial or complete visual loss, acute and subacute motor dysfunction of the limbs , diplopia, and gait dysfunction are commonly found.[5].
Adult polyglucosan body disease (APBD) It is characterized by adult onset progressive neurogenic bladder, gait difficulties , spasticity and weakness. Mixed upper and lower motor neuron involvement can be found. Sensory involvement predominantly seen in the distal lower extremities. There may be associated mild cognitive difficulties.[6].
Myasthenia gravis (MG) In MG bulbar involvement usually present but not always. It is associated with fatiguability, diplopia and ptosis. There is no wasting or UMN signs.[7].
Cervical spondylosis Cervical spondylosis with cervical stenosis causing UMN signs in the legs and LMN signs in the arms should be considered as a differential diagnosis. Cervical spondylosis also commonly found in patients with ALS.[8].Progressive limb weakness can be seen but often stabilises or variably progressive. It has asymmetrical onset with combined UMN and LMN signs in arms. Spastic paraparesis can also be seen. Fasciculations in arms can found occasionally.[7].
References
  1. PRIOR TW, RUSSMAN BS, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Spinal Muscular Atrophy [online] 1993 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301526
  2. LA SPADA A, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Spinal and Bulbar Muscular Atrophy [online] 1993 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301508
  3. FINK JK, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Hereditary Spastic Paraplegia Overview [online] 1993 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301682
  4. KABACK MM, DESNICK RJ, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Hexosaminidase A Deficiency [online] 1993 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301397
  5. STüVE O, OKSENBERG J, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Multiple Sclerosis Overview [online] 1993 [viewed 16 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301492
  6. KLEIN CJ, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Adult Polyglucosan Body Disease [online] 1993 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301758
  7. LEIGH P, ABRAHAMS S, AL-CHALABI A, AMPONG M, GOLDSTEIN L, JOHNSON J, LYALL R, MOXHAM J, MUSTFA N, RIO A, SHAW C, WILLEY E. THE MANAGEMENT OF MOTOR NEURONE DISEASE J Neurol Neurosurg Psychiatry [online] 2003 Dec, 74(Suppl 4):iv32-iv47 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.74.suppl_4.iv32
  8. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 17 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623

Investigations - for Diagnosis

Fact Explanation
Full blood count (FBC) At present there are no specific biochemical or pathological markers for the detection of MND.[1]. It is diagnosed by Clinical history and examinations, findings on electrodiagnostic testing and exclusion of other possible diseases.[2]. Laboratory investigations are mainly focused on exclusion of other DD's and thereby supporting the diagnosis.[1]. FBC is an essential investigation to be carried out.[3].
Erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) Essential investigations,should be carried in all patients with suspected MND to exclude other inflammatory causes.[1],[3].
Creatine kinase Elevated levels of serum creatinine kinase can be seen predominantly in lower motor neuron involvement and slow progression.It can also found in Kennedy's disease.[1].
Electromyogram (EMG) It can show electrophysiologic evidence of LMN involvement in clinically involved or uninvolved regions.[2].There are EMG criteria for diagnosis of motor neuron disease such as Fibrillation and fasciculation in muscles of the extremities, or both extremities and head, Increase in amplitude and duration as well as reduction in number of motor unit action potentials, normal conduction velocity and excitability in relatively unaffected motor fibers and normal conduction velocity and excitability seen even in sensory fibers of severely affected extremities. [1].
Nerve conduction studies Carried out in patients to detect sensory and motor involvement. It also useful in exclude other neuromuscular disorders such as myopathy and motor neuronopathy.[1]
Muscle or nerve biopsy Carried out to evaluate other health conditions with related symptoms if indicated. However the definitive diagnosis of ALS can be made based on brain stem and spinal cord pathology.[2].
Genetic studies Familial amyotrophic lateral sclerosis can be inherited in an autosomal dominant, autosomal recessive, as wll as X-linked manner.Molecular genetic testing is carried out to detect mode of inheritance.[2].
Lumbar puncture and CSF analysis Used in atypical cases.The presence of oligoclonal bands, increased numbers of leucocytes, or elevated level of protein can be seen in underline other disease.[1]. Further more CSF examination will rule out infection and multiple sclerosis.[2].
Anti-ganglioside (GM1) antibodies There are many conditions that can mimic MND. Antibodies against gangliosides carried out in such occasions.[1]. These are sometimes elevated in autoimmune motor neuropathies.[2].
Anti-neuronal antibodies Carried out to exclude other associated diseases. Serum neuronal autoantibodies for paraneoplastic syndromes found to be associated with occult cancer.[2],[3].
MRI Many neurologist suggest that MRI is mandatory in suspected case of MND to exclude possibility of signs occurring due to single lesion. MRI mainly focus on head, neck,or thoracolumbar region, depending on the presenting symptoms and signs. It is useful to exclude spondylotic myelopathy or neoplasm. In MRI of MND, bilateral T2 shortening in the precentral cortex and hyperintensity of the spinal cord corticospinal tracts can be found.[1].
References
  1. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  3. LEIGH P N. The management of motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 December, 74(90004):32iv-47 [viewed 15 September 2014] Available from: doi:10.1136/jnnp.74.suppl_4.iv32

Investigations - Fitness for Management

Fact Explanation
Vitamin B12 and folate levels Pure upper motor neuron syndrome can be occurred due to vitamin B12 deficiency. So that this investigation is carried out to detect those nutritional deficiencies.[1],[3].
Chest x ray Essential investigation to be performed to exclude other chest pathlogies.[1],[4].
Blood lead and 24-hour urinary lead excretion. Testing of heavy metals is carried out if exposure is suspected.[1],[2].
HIV and HTLV1 assay HIV is taken as a risk factor for development of mixed upper and lower motor neurone syndrome. So that these investigations are carried out.[1],[3].
Thyroid function tests and Autoantibody screen Performed in the purpose to exclude thyroid and parathyroid disorders as well as autoimmune disorders. Because they may be associated with motor neuronopathy or atypical spinal cord syndromes.[1].
VDRL test Carried out as a basic screening investigation to detect associated syphilis infection.[1],[2].
Single-photon emission computed tomography (SPECT) Not widely used. It will show reduced cerebral blood flow in the frontal regions in patients with MND and dementia.[1].
References
  1. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  3. TALBOT K. Motor neurone disease Postgrad Med J [online] 2002 Sep, 78(923):513-519 [viewed 15 September 2014] Available from: doi:10.1136/pmj.78.923.513
  4. LEIGH P N. The management of motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 December, 74(90004):32iv-47 [viewed 15 September 2014] Available from: doi:10.1136/jnnp.74.suppl_4.iv32

Investigations - Followup

Fact Explanation
Pulmonary function tests It is suggested to carried out specialized multidisciplinary ALS clinic evaluations in every three months. There pulmonary function tests should be performed. At the same time neurological evaluation ,speech and nutritional evaluation should be carried out.
References
  1. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623

Investigations - Screening/Staging

Fact Explanation
Diagnostic criteria for motor neuron disease The diagnosis of ALS requires the presence of LMN signs including EMG features in clinically normal muscles, UMN signs as well as Progression of the disorder.[1]. It can further categorized as follows. 1.Definite ALS Should have UMN signs and LMN signs in three regions. 2.Probable ALS Should have UMN signs and LMN signs in two regions with at least some UMN signs rostral to LMN signs. 3.Probable ALS: laboratory supported Include UMN signs in 1 or more regions and LMN signs defined by EMG in at least two regions. 4.Possible ALS There should be UMN signs and LMN signs in one region (together), or UMN signs in two or more regions or UMN and LMN signs in two regions with no UMN signs rostral to LMN signs.[2].
References
  1. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069
  2. LEIGH P N. The management of motor neurone disease. Journal of Neurology, Neurosurgery & Psychiatry [online] 2003 December, 74(90004):32iv-47 [viewed 17 September 2014] Available from: doi:10.1136/jnnp.74.suppl_4.iv32

Management - General Measures

Fact Explanation
Therapy for swallowing impairment Treatment is mainly palliative and it should be mainly by multidisciplinary team approach, including: a neurologist, specially trained nurses, pulmonologist, speech therapist, physical therapist, occupational therapist, respiratory therapist, nutritionist, psychologist, social worker, and genetic counselor.[1]. In order to improve swallowing mechanism they should be feed in a normal physiological position and should be supported.compensatory and restitutional swallowing therapeutic methods can be carried out. Even those method may not show marked improvement in swallowing but useful to prevent food aspiration.Tilting head backwards to support the transport of the food bolus by gravity is useful method in patients with tongue weakness.Diet can be change in to a fluid or moist consistency if there are any mastication problem.Patients should be encouraged to seal their lips with their hand in order to prevent drooling while feeding.Sensorimotor exercises is a restitutional swallowing therapeutic method that can be used.Thermal stimulation of the anterior faucial pillars and the tongue before meal and Passive pressure on the hyoid bone during eating can be carried out to trigger the swallowing reflex. [2].
Dilatory modification Asked to eat as several small meals per day. Further more they should avoid from surrounding distractions and should be focus on meal.Heimlich maneuver can be performed if patient develop chocking attack. Dieticians advice should be taken regarding preparation of meals.It should be high in calories, proteins and vitamins.Thickened fluids are usually better tolerated than thin fluids in case of swallowing difficulty.Special eating or drinking aids can also be used. More fibers can be added to the diet in case of constipation.[2].
Use feeding tubes Short term feeding can be carried out with the help of nasogastric feeding tubes. They may reduce frequent chocking and reduces prolong meal time but they may carry a risk of ulceration and aspiration pneumonia. Percutaneous endoscopic gastrostomy (PEG) or percutaneous endoscopic jejunostomy can be used for enteral feeding. [2].
Speech theraphy Physiotherapists or occupational therapists is consult in order to maintain body position and respiration. The background noise should be avoided.Breathing and relaxation exercises should be taught. To decrease muscle tone vibration, or application of ice to the involved musculature can be performed. Augmentative and alternative communication devices have to be used in case of progressive dysarthria.[2].Low-tech such as alphabet board and high-tech such as computer-assisted devices can aid speech and communication.Eye movement-controlled on-screen keyboard has been recently developed to improve communication.[1].
Prevention and treatment of laryngospasm Upright positioning of the trunk is useful to reduce duration of laryngospasm. Appropriate spacing of meals and avoidance of late-night meals may useful in long term management.[2].
Prevent drooling of saliva Neck support and correction of the head position may helpful. Mouth infections should be treated and family members should be educated that this is a manifestation of disease itself and not due to mental impairment.[3].
Management of respiratory failure Correct positioning, specialy during sleep; frequent turning at night; and prevention of aspiration and removal of secretions with a hand operated or electrical suction machine as well as regular monitoring of forced vital capacity should be done. Continuous positive airway pressure ventilation can be perform in case of dyspnea. Tracheostomy can be performed in case of emergency.[3].
Management of peripheral oedema Elevation of legs, elastic stockings can be done.Diuretics can be use with caution.[3].
Management of sleep disturbances Sleep disturbances can be overcome by taking simple measures such as providing comfortable bed at night.Beds should be in correct height with a comfortable mattress. Turning beds with powered elevator has an added advantage.[3].
Provide assistive devices Walkers or wheelchairs, can aid mobility. To improve activities of daily living at home Hoyer lift, hospital bed, and bathroom installments can be provided.[1].
Proper counselling with patient and family education. Done as a multidisciplinary team approach.Once diagnosis is established proper counselling should be carried out. Braking bad news should take place with privacy, and a provisional plan for early follow up. Further support should be agreed. Educate the patient and family members about the disease characteristics and tell them that patient needs their helping hand to do their day today works and get rid of their stress. Educate them that this is a long term disease and can leads to further complications, so that caring, keeping them happy and proper follow up is needed. Follow up clinic visits should be arranged every 2 or 3 weeks.[3],[1].
Genetic counseling Genetic counseling is the process of providing individuals and families with information on the nature, inheritance, and implications of genetic disorders to help them make informed medical and personal decisions. This should be carried out because disease has tendency to spread in several inheritance patterns,including autosomal dominant, autosomal recessive, or X-linked manner.[1].
References
  1. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623
  2. KüHNLEIN PETER, GDYNIA HANS-JüRGEN, SPERFELD ANNE-DORTE, LINDNER-PFLEGHAR BEATE, LUDOLPH ALBERT CHRISTIAN, PROSIEGEL MARIO, RIECKER AXEL. Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis. Nat Clin Pract Neurol [online] December, 4(7):366-374 [viewed 15 September 2014] Available from: doi:10.1038/ncpneuro0853
  3. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069

Management - Specific Treatments

Fact Explanation
Riluzole It is only currently FDA-approved drug for the treatment of ALS. It act by glutamate inhibition. [1].Riluzole modulates glutaminergic transmissions by presynaptic inhibition of glutamate release and postsynaptic interference with the effects of excitatory amino acids. The effect of riluzole was found to be greater in patients with bulbar onset compared with limb onset MND.[2]. But it was found to be associated with elevation of serum alanine aminotransferase as well as bone marrow supression.[1].
Reduce oral secretions Tricylic antidepressants and anticholinergic agents can be use to reduce oral secretions in patients with bulbar symptoms.It will reduce the need of suction.[1].
Relieve spasticity Benzodiazepines, baclofen, or can be used. But they have side effects such as weakness and lethargy.[1]. Surgical lengthening- or division of soft tissues under local or general anesthesia may helpful in severe contractions.[2].
Reduce pain Other than non pharmacological management such as correct positioning of the patient,physiotherapy and use of a turning bed at night there are several pharmacological treatments. Drugs such as benzodiazepines and baclofen causes muscle relaxation, thereby relieve pain. In advanced stage of the disease non-steroidal antiinflammatory drugs, and opioids can be prescribed.[2].
Treatment for constipation Bulk-forming laxatives such as methyl cellulose, osmotic laxatives such as lactulose; suppositories and enemas like pharmacological measures can be taken other than dietary measures to overcome constipation.[2].
Antidepressant Antidepressant drugs can be used in individual basis for emotional problems such as anxiety and depression.Some patients get benefits from imipramine.[1].
References
  1. LEIGH PN, RAY-CHAUDHURI K. Motor neuron disease. J Neurol Neurosurg Psychiatry [online] 1994 Aug, 57(8):886-896 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1073069
  2. KINSLEY L, SIDDIQUE T, PAGON RA, ADAM MP, ARDINGER HH, BIRD TD, DOLAN CR, FONG CT, SMITH RJH, STEPHENS K. Amyotrophic Lateral Sclerosis Overview [online] 1993 [viewed 15 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301623