Dr Meera Narendran
Motor neuron diseases are characterized by selective degeneration of motor neurons, both upper and lower, namely of the pyramidal fibers in cerebral cortex, of ventral horn cells and of cranial motor nerve nuclei. In different cases relative incidence of degeneration varies. In some the change is maximal in the ventral horn cells, in others in the cranial nerve nuclei, in others pyramidal fiber sclerosis is severe and adds the appropriate clinical picture.
Aeteology: – About 5 % of cases are familial, showing autosomal dominant inheritance. In many such families genetic defects lies on chromosome 21, the enzyme involved being a superoxide dismutase (SOD1).
For the remaining 95 %, probable causes includes
1. Chronic Aluminium toxicity
2. Slow virus infection
3. Auto immunity
5. Electrical shock
Another suggestive hypothesis is that glutamate which is a primary excitatory neurotransmitter in the CNS, accumulates at synapses and causes the neurons to die, probably through a calcium dependent mechanism. Prevalence of this disease is about 5/100000.
The motor neurons in the cerebral cortex, brainstem and spinal cord show atrophy and their axons show degenerative changes. Muscles show groups of atrophic fibres amidst the groups of normal fibres.
A. Classical type and
B. Non classical type
A. Classical type:
1. Predominant LMN involvement
a) Bulbar form: Progressive bulbar palsy
b) Spinal form: Progressive muscular atrophy
2. Predominant UMN involvement
a) Bulbar form: Pseudo bulbar palsy (spastic bulbar palsy)
b) Spinal form: Primary lateral sclerosis.
3. Combination of upper and lower motor involvement.
Amyotrophic lateral sclerosis.
B. Non classical type:
1 Werding – Hoffmann disease
2 Kugelberg – Welander disease
3 Spinal muscular atrophy described from south India (Madras )
4 Motor neuron disease – dementia – parkinsonian complex described from Guam Island
Clinical feature of motor neuron disease
Patient s present with a combination of lower and upper motor neuron signs with out sensory involvement. The presence of brisk reflexes in wasted limb muscles is typical
Common presenting features are
Age of onset
Usually after age 50 years
Very uncommon before 30 years
Males are common than females.
Limbs muscle weakness, cramps, occasionally fasciculation.
Disturbance of speech and swallowing (dysarthria and dysphagia )
Wasting and fasciculation of muscles
Weakness of muscles of limbs and tongue, face and palate.
Pyramidal tract involvement causes spasticity, exaggerated tendon reflexes and extensor plantar responses.
External occular muscles and sphincters usually remain intact.
No objective sensory deficit.
No intellectual impairment in most cases.
Symptoms often begin focally in one part and spread gradually but relentlessly to become widespread.
There are two types:
1. Progressive bulbar palsy
2. Pseudobulbar palsy
Apart from the causes mentioned earlier, there are certain other causes leads to these types of palsies. They are:
|Pseudobulbar palsy||Bulbar palsy|
|Genetic||Kennedy’s disease (x linked bulbospinal neuropathy|
|Vascular||Bilateral hemisphere (laccunar ) infarction||Medullary infarction|
|Inflammatory /Infective||Multiple sclerosisCerebral vasculitis||Myasthenia
Guillain – Barre
|Neoplastic||High brainstem tumors||Brain stem glioma
Progressive Bulbar Palsy
This is the most serious form since it terminates life within a period of one year. There are spastic and atrophic variants of chronic bulbar palsy. In a very few cases the spastic element so far predominates that little wasting may be seen. In some atrophic element is predominating, evidenced by wasting of muscles of tongue, muscles of mastication and face. There is progressive involvement of palatal pharyngeal and laryngeal muscles. Usually patient seeks advice for a slowly increasing difficulty in articulation, shortly followed by difficulty in deglutition. On examination, fasciculation can be seen in the muscles in the region of tongue and chin. As the malady progresses, the lips are slightly open and saliva trickles from the corners of the mouth. Patient can not purse the lips and whistle.
The mucous membrane of the tongue may be wrinkled. The range of movement of the tongue is limited and the protrusion beyond the lips may be impossible. On phonation, the excursion of soft palate is diminished. Swallowing is slow and laboured because of the weak muscles of the tongue and floor of the mouth. Patient can not push the food bolus back into the fauces and pharyngeal deglutition is impaired owing to the weakness of the hyoid muscle. The larynx is not fixed, moves up and down. The nasopharynx is not shut off. Articulation is grossly disturbed and the voice nasal in quality and monotonous. Finally, the patient can make only inarticulate noises, swallowing becomes virtually impossible and aspiration pneumonia results. This will lead to termination of life within one or two years of the time of onset.
When the spastic element is prominent, the face moves as a whole, slowly and in exaggerated degree and there may be tendency to spasmodic weeping and laughter of the type found in pseudobulbar palsy, with which this variant of chronic bulbar palsy may be confused if the fact of its slowly progressive onset be overlooked. In pseudobulbar palsy the onset is usually sudden after two or more cerebral ischaemic attacks. Chronic bulbar palsy may also occur along with amyotrophic lateral sclerosis.
This results from small bilateral softening in the region of internal capsule. There is usually a history of two or more mild apoplectiform seizures, neither of which has been followed by severe hemiplegia. In this form of palsy affection of bulbar muscles is of UMN type. There is weakness of bulbar muscles. The palatal muscles, tongue and pharyngeal muscles are spastic. There is dysarthria, sometimes so gross as to render the patient’s speech unintelligible. The tongue is small and spastic and usually can not be protruded beyond the line of teeth. Swallowing and mastication are laboured and imperfect. The lips hang apart and saliva trickles from between them. Palatal movements are slow, but the gag reflex is brisk. Jaw jerk is exaggerated. Emotional movements of the face are apt to be grossly exaggerated, the whole face being contorted in to smile or into an expression of woe. Spasmodic and uncontrollable laughter or weeping occurs from time to time. The limbs show some measures of spasticity. Fibrillation of the tongue may occur in later stage.
Amyotropic Lateral sclerosis
This is the most common type. Important features are:
Combination of distal and proximal muscles wasting, weakness and fasciculation.
Spasticity, exaggerated reflexes and extensor plantar reflexes.
Bulbar and pseudobulbar palsy follow eventually.
Pyramidal tract features may predominates .
Progressive muscular atrophy: Weakness and wasting starts in one upper extremity (75%) or one lower extremity (25%) and this become generalized over a period of 3-5years. Fasciculation are visible. Despite the marked wasting, reflexes are preserved normally or may even be brisk.
Primary lateral sclerosis: UMN signs in the lower limbs with LMN signs in upper limbs.
Variant Types Of MND:
Werding-Hoffmann disease: This form of progressive muscular atrophy is seen in infancy and runs a more acute course.
Kugelberg-Welander disease: This is one of the form of spinal muscular atrophy characterized by affection of the proximal muscles of all four limbs. Starting at adolescence and has a benign course. It follows a chronic course.
Other Forms Of Spinal Muscular Atrophy: These occur at any age after infancy. They manifest as asymmetrical uni. or bilateral weakness and wasting of muscles with predominantly LMN features.
Geographic Variant (Madras form):Weakness and wasting may be uni or bilateral, proximal or distal, and asymmetrical. Bulbar muscles may be involved in 60% of cases. Uni. or bilateral nerve deafness is an associated feature.
Another Variant Of MND Described From Guam Island: Demonstrates the picture of ALS, parkinsonism, and dementia.
Diagnosis: MND has to be suspected under the following circumstances:
1) Insidious onset of selective motor system involvement characterized by affection of the lower and upper motor neurons.
2) Weakness, atrophy of muscles and fasciculation
3) Electromyographic abnormalities as
a) fibrillation and fasciculation at rest
b) reduction in the number of motor unit potentials
c) giant potentials with increased duration and amplitude
4) Sensory nerve conduction and motor nerve conduction studies are normal, but there is evidence of loss of axons.
5) Spinal imaging and brain scanning may be necessary to exclude focal spinal or cerebral disease.
6) CSF examination is usually normal, but a slight elevation of protein concentration may be found.
Motor neuron disease is progressive. Mean time from the diagnosis to death is one year. Most of the patients dying within 3-5 years of onset of the disease symptoms.
Younger patients and those with early bulbar symptoms tend to show more rapid course.
Death is usually due to respiratory infections, respiratory failure and the complication of immobility.
1) Carcinomatous neuropathy resembles MND.
2) Diabetic amyotrophy – Wasting of proximal muscles of lower limbs. This is correctable with proper control of diabetes.
3) Syphilitic amyotrophy – Wasting of muscles of the hands with UMN lesions of lower limbs.
4) Cervical cord compression occurring in tumors and cervical spondylosis- Wasting And spastic paralysis of lower limbs. Sensory manifestations are also present.
5) Myopathies and chronic polymyositis – Presented as muscle wasting and atrophy. In these fasciculations are absent. Muscle biopsy is diagnostic. Muscle enzyme CPK is elevated. Myopathy will help to distinguish from MND.
At present, no treatment significantly arrests the progress of the disease.
Palliative measures includes:
1) Psychological supports
2) Physical supports with the help from occupational therapists, speech therapists and physiotherapists.
These measures are essentials to keep the patient’s quality of life as good as possible.
3) Mechanical aids like splints walking aids, wheelchairs and communication devices all helps to reduce the handicap.
4) Feeding by percutaneous gastrostomy may be necessary if bulbar palsy is marked.
5) Non invasive ventilatory support may help the distress from weak respiratory muscles.
6) In allopathic system, they use the drugs that modulate glutaminergic system (Glutamate antagonists like riluzole ). And agents such as nerve growth factors.
Homoeopathic Management: – Similimum.
I. Repertory of William Boerick:
1) Nervous system, Bulbar paralysis: Guaco, Plumb. met, Mang.oxydatum.
2) Nervous system, Degeneration(softening, sclerosis):
2+ – Aur.mur,Phos, Plumb.met.
1+- Alum, Alum.sil, Arg.nit. Aur, Bar.mur, Carb.sulph, Naja, Oxalic.acid, Phos, Physostigma, Picric .acid.
II. Kent’s repertory:
1) Throat, swallowing, impossible, paralysis from:
2+- Alum, Alumn, Apis, Cocc, Gelse, Nat. mur, Nux Vom,Tab
2)Mouth, speech, wanting paralysis of organs from:
2+- Anac, Crot.c, Gelse, Glon, Mur.acid, Staph.
3) Mouth, speech difficult:
3+- Bell, Crot.c, Gels, Lach, Nat.mur, Op, Stann.
4) Throat,liquids taken are forced in to nose:
3+- Arum.t, Lach, Lyc
2+- Bar. carb, Carb.ac, Cur,Nat.mur,Phyt,Plumb.
III. Rau’s special pathology:
1) Paralysis of bulbar muscles: Caust, Hyos,Nux.vom,Cocc,Gels, Op,Plumb,Ruta
2) Paralysis of face:Bell,Caust,Cocc, Graph, Nux vom
3) Paralysis of tongue and organs of speech:Arn, Acon, Ars,Bar.carb, Bell, Caust, Cocc, Cupr, Dulc, Lach, Op, Mur.ac, Plumb, Hyos,Stann.
IV. Boeninghausen’scharacteristicmateria medica and repertory:
1) Mouth, throat and gullet, paralysis of deglutition
3+- Cocc, Gels, Laur, Lach
2) Voice and speech, paralysis of vocal cord:
3+- Caust, Hyos,Laur,Nux vom, Rhust, Stram.
Causticum: Paralysis of single parts- vocal organs, tongue, eyelids, face, bladder, extremities, generally of rt sided. Paralysis from exposure to cold wind or draft. Paralysis after typhoid, typhus or diphtheria;appearing. Drooping of eye lids,cannot keep them open. It is used in paralysis which is remote from apoplexy, the paralysis remaining after patient has recovered from apoplexy with inability to select proper words. Laryngeal muscles refuse their services, cannot speak a loud word. Aphonia. Sudden aphonia after taking cold. Paralysis of face or tongue or hemiplegia with giddiness, weakness of sight, weeping mood, hopelessnessand fear of death.
Guaco:-Acts on nervous system. Bulbar paralysis. Deafness. Tongue heavy and difficult to move. Spinal irritation.Spinal symptoms are most marked. Beer drinkers threatened with apoplexy. Larynx and trachea are constricted. Difficult deglutition. Paralysis of lower extremities.
Plumbum metalicum: Paralysis with atrophy. Muscular atrophy from sclerosis of spinal system. Excessive and rapid emaciation. General or partial paralysis with great weakness and anaemia. Clonic ortonic spasm from cerebral sclerosis or tumor. Paralysis of plumbum is prominently of spinal origin. Paralysis of upper extremities is more marked. Ptosis. Heavy tongue. Difficulty in articulation. Tremor of nasolabial muscles. Twitching of the side of the face. Paralysis of gullet and inability to swallow. Paralysis of lower extremities with paralysis of single muscle. Paralysis from over exertion.
Manganum oxydatum: Low monotonous voice. Economical speech. Mask like facies. Muscular twitching. Cramps in calves Stiff leg muscles. Occasional uncontrollable laughter. Peculiar slapping gait. Workers of manganum binoxide are frequently affected with bulbar paralysis. Pseudo sclerosis. Progressive lenticular degeneration. Similar symptoms to paralysis agitance.
Plumbum iodatum: Has been used empirically in various forms of paralysis. Sclerotic degeneration, especially of spinal cord. Atrophies.
Phosphorus: Paralysis from fatty degeneration of nerve cells. Progressive spinal paralysis. Ascending sensory and motor paralysis from ends of fingers and toes. Arms and hands become numb. Fingers feel like thumb.Can lie on right side. Post diphtheritic paralysis. Tottery gait Periodical contractions of fingers as from cramps.
Cocculus: Paralysis of facial nerve especially of one side. Or tongue pharynx. Paraplegia and rheumatic lamness in weakened or nervous subjects, who are inclined fainting fits and palpitation of the heart. Paralytic affection originates in the small of the back after taking cold, with cold feeling of extremities and edema of the feet. Paralysis after apoplexy. Paralysis of lower limbs. Paralytic immobility. One sided paralysis of the face with cramp like pain in masseter < opening the mouth. Prosopalgia. Tremor of lower jaw and chattering of teeth when attempting to speak. Lines of face are deepened as if drawn. Paralysis of the tongue with difficult speech. Pains at the base of the tongue when protruded. Paralysis of muscles of deglutition with difficulty to swallow.
Gelsemium: Complete motor paralysis, rather functional than organic in nature. Paralysis of occular muscles. Ptosis. Paretic condition of the tongue causes difficulty to speak. Speak is thick. Paralysis from emotions. Post diphtheritic paralysis. Paralysis of larynx causes aphonia. Locomotor ataxia. Paraplegia.
Aconitum nepellus: Facial paralysis accompanied with coldness from exposure to dry cold wind especially in acute cases. Paraplegia with tingling. Numbness of the affected parts.
Lachesis: Especially left side. Awkward. Stumbling gait. Paralysis after apoplexy. Trifacial neuralgia. Spasm of glottis.
Nux vomica: Incomplete paralysis of the face, arms, and legs with vertigo, weak memory, darkness before the eyes, ringing in ears, loss of appetite, burning in stomach, flatulence and vomiting after eating and drinking.Constipation especially in drunkards. Jaw contracted. Infra orbital neuralgia. Left angle of the mouth drops. Twitching and spasmodic distortion of face. Articulation and speech difficult. Paralysis of arms. Automatic motion of hand towards mouth.
Opium: Paralysis and insensibility after apoplexy, in drunkards, in old people, associated with retention of stool and urine. Spasmodic facial twitching, especially of corners of mouth. Hanging down of lower jaw. Distorted face. Twitching of facial muscles. Face covered with profuse sweat. Paralysed tongue which dry and black. Difficult articulation and swallowing. Tongue protrudes to right side. Inability to swallow. On swallowing food goes the wrong way or returns through nose. Painless paralysis. Twitching of limbs. Numbness. Jerks as if flexors are overacting. Sensation as if lower limbs were severed and belongs to someone else. Shifting and trembling gait. One or other arm moves convulsively to and fro. Coldness of extremities.
Stramonium: Paralysis after convulsion,. Paralysis of one or spasm of other side. Stammering speech. Cannot swallow on account of spasm.
Graphitis: Rheumatic, peripheric paralysis of face. Distortion of muscles of face and difficult speech. Sensation of cobweb over the face.
Belladona: Apoplexy , congestion of the head, paralysis of one and spasm of other side of the body, paralysis of the face and locomotor ataxia.
Arnica: Paralysis due to exudation within the brain or spine. Paralysis in consequence of apoplexy, of concussion, of weakening disease and of protracted intermittent fevers. Paralysis of face and lower lip hang down. Lower lip trembling while eating.
Conium: Paralysis from periphery upwards, of old women. Speech difficult from paralysis of tongue. Distortion of tongue and mouth. Food goes down the wrong way and stops while swallowing. Paralysis of lower limbs than of upper limbs. Staggering < turning the head or looking sideways.
Arsenicum album: Paralysis associated with great prostration and neuralgic pains. Spinal affection with gressus gallinaceus. Twitching of muscles of face. Paralysis and contraction of limbs.
Ruta graveolance: Facial paralysis after catching cold.
Baryta carbonicum: Causes paralysis by producing degeneration of the coats of the blood vessels. Facial paralysis. Paralysis of old people. Paralysis after apoplexy. Facial paralysis of young people where the tongue is implicated.
Natrum muriaticum: Paralysis from cold. Numbness. Tingling of tongue and lips. Loss of taste. Tongue striped along the edge. Numbness and stiffness ofone side of the tongue. Tongue heavy and difficult speech. Tongue feels dry but actually not dry. Uvula hangs to one side. Food goes down the wrong way. Post diphtheritic paralysis. Fluids can be swallowed. Paralytic condition of lower limbs.
Curare: It is a great remedy for paralysis of various kinds and of various parts of our body. General paralysis of motor system. Ptosis. Facial and buccal paralysis. Paralytic failure of power to swallow. Paralysis of deltoid muscles.
1. Principles and practice of medicine– Davidson
2. Text book of medicine- K.V. Krishna Das
3. Disease of nervous system- Sir. Francis Walshe
4. Disease of nervous system- W.B.Mathews and Henry Miller.
5. Brain’s disease of the nervous system- Sir John Walton.
6. Harrison’s Principles of internal medicine
7. Repertory of William Boerick
8. Kent’s Repertory.
9. Boeninghausen’s Characteristics Materia Medica and Repertory.
10. Raue’s Special Pathology.