1

A CLINICAL APPROACH TO FLACCID QUADRIPARESIS/ QUADRIPLEGIA

L.J Basumatary, MD,DM neuro trainee,GMCH,guwahati mailto:[email protected] Definition: Weakness means a reduction of normal power of one or more muscles. Paresis means weakness that is mild to moderate. Plegia means weakness that is so severe that it is complete or near complete. Quadri- refers to all four limbs. So if all the four limbs are having paresis or plegia it is said as quadriparesis or quadriplegia. Tone: It is defined as a state of partial sustained contraction of muscle. It is of two type hypertonic and hypotonic states. Hypertonic state is caused by Upper motor neuron type of palsy and hypotonic state is caused by Lower motor neuron type of palsy, Upper motor neuron type of palsy in shock state and myopathies. Proposed sites where lesion can cause flaccid quadriplegia. UMNP in shock Anterior horn cells. Ventral or motor root. Spinal nerve. Peripheral nerve. Neuromuscular junction. Muscle.

2 Associated symptoms: Upper motor neuron palsy in shock: These diseases always have an acute onset and there is loss of consciousness. The diseases are With loss of consciousness: Cerebrovascular accident Mid brain, pons and Medulla. Following seizureo Partial seizure with secondary generalization. o Generalised tonic clonic seizure Following head injury. Poisoning. Diabetic and hypoglycaemic comas. Uraemic coma. Hepatic coma. Myxoedematous coma.

Without loss of consciousness: Upper cervical cord transverse myelitis. Upper cervical cord trauma.

Anterior horn cell disease: These diseases usually begins proximally but may present distally and typically asymmetric initially and is not associated with numbness and fasciculations are visible. The diseases acting here are Spinal muscular atrophy infantile and chronic childhood type. Poliomyelitis.

Ventral or motor root or Spinal nerve disease:

3 Landry Guillain Barre syndrome.

Peripheral nerve disease or peripheral polyneuropathy: These disease generally present distally first then involves proximally; onset can be acute, sub acute or chronic and sensory system involvement is generally found. The diseases acting here are

Acute idiopathic demyelinating polyneuropathy: o Guillain Barre Syndrome o Acute motor axonal neuropathy o Acute motor sensory neuropathy. o Miller Fischer syndrome.

Neuropathy due to infection: o HIV. o West Nile Fever.

Neuromuscular junction disorders: These diseases present as weakness and fatigability of skeletal muscles. On repeated usage there can be incremental or decremental effect in the muscle power. The diseases acting here are Myasthenia gravis. Botulism.

Muscle disorders or myopathies: These diseases generally present as proximal weakness of the four limbs, onset can be acute, sub acute or chronic. The diseases acting here are

Disorders of muscle membrane exitability. o Hypokalemic periodic paralysis.

Endocrinal and metabolic myopathies.

4 o Thyroid disorders. o Adrenal disorders.

Evaluation of the quadriparesis quadriplegia patient. Through detailed history taking. Through meticulous clinical examination. Through appropriate investigations.

HISTORY:

The following points are to be evaluated from the history of the patient. 1. Whether the patient is truly having quadriparesis or quadriplegia? A patient with bilateral brachial paresis/palsy will complain of difficulty/inability in doing all types of function that is done by the upper limbs e.g. feeding, dressing, combing. A patient with paraplegia will complain of difficulty/inability of walking, sitting, standing. So a combination of these two will suggest quadriparesis or quadriplegia. 2. If there is quadriparesis whether it is flaccid or spastic? If the patient is having spastic quadriparesis/quadriplegia there will be difficulty in passive movement of the joints and the attendants will have difficulty in maneuvering the patient in the bed, vehicle and will find it difficult to bring the patient to the OPD or casualty dept. But if he or she is having flaccid type of paresis or plegia it will be easier as the patient could be easily mouldable to the space present. 3. Whether the patient is conscious or not?

5 If there is history of indifference to the surroundings, non-responsiveness to external stimuli like verbal commands, painful stimuli, involuntary passage of stool or urine it suggests loss of consciousness and generally suggests UMNP in shock. If there is no loss of consciousness it signifies pathology involving the LMN or UMNP with only spinal shock. 4. What is the mode of onset? If the onset is acute in nature with loss of consciousness then it is a UMNP in shock but if there is no loss of consciousness then it could be a case of spinal trauma above C5 level in spinal shock, Myasthenia gravis or acute poliomyelitis. If the onset is gradual it signifies diseases like auto-immune diseases (AIDP), metabolic disorders, hormonal disorders. If the onset is chronic then it is a chronic pathology and signifies diseases like congenital diseases (Type I, II, III Hereditary motor and sensory neuropathy), hereditary diseases (Duchenne muscular dystrophy) and autoimmune diseases like CIDP. 5. What is the extent of disability? Treatment modalities will differ with the degree of disability. If a patient is having quadriparesis adequate changes in his home is to be made e.g. the patient is to live in the ground floor, if that is not possible railings are to be fixed, change in his bathroom settings like European type of Kumod adequate place to hold is to be used. If a patient is completely in able to use his limbs there should be frequent change of body position and proper care of his hygiene is required. The joints of the body should be passively mobilized. If swallowing is not possible adequate feeding via a Ryles tube or gastrostomy tube is to be started. 6. Whether the bladder or bowel is involved? When the bladder or bowel is involved it signifies that the spinal cord is in shock state alone, if there is no loss of consciousness and can occur in spinal trauma above C5 level, Guillain Barre

6 syn, and acute poliomyelitis but if there is loss of consciousness the whole of the CNS is in shock. 7. Is there any associated respiratory difficulty? If there is associated respiratory difficulty then the phrenic nerve is also involved. If it is due to spinal trauma then the lesion is above or at C3 level in spinal shock. Other causes are Guillain Barre Syndrome and acute poliomyelitis. 8. Where is the site of initiation? If the lower limbs are the first to be involved then it is an ascending type and if the upper limbs are the first to be affected then it is a descending type of palsy. If it is of ascending type generally the diseases are acute Poliomyelitis, Guillain Barre Syndrome. Sometimes though rare Guillain Barre Syndrome can present as a descending type. If all the limbs are involved simultaneously then theses are diseases causing polyneuropathy, myopathy or neuromuscular junction disorders. 9. Disability is more proximally or distally? Predominant proximal weakness patients will complain of difficulty in sitting, standing or getting up from squatting position, dressing, undressing and bringing the food to the mouth. Predominant proximal muscle weakness is caused by myopathies and anterior horn cell diseases. Predominant distal weakness patients will complain of mashing the food during feeding, writing, walking with chapples and hitting the ground during walking. Predominant distal weakness is caused due to peripheral polyneuropathy and rarely anterior horn cell diseases or myopathies. 10. Onset is symmetrical or asymmetrical? If there is involvement of both sides of the Sagittal plane simultaneously like simultaneous involvement of both the lower limbs or the upper limbs, then the onset is symmetrical. The

7 diseases causing myopathy or acting in the neuromuscular junction generally has a symmetric onset. If the involvement is not simultaneous in both sides of the Sagittal plane i.e. the upper or the lower limbs are not involved simultaneously, then the onset is asymmetrical. The diseases acting in the anterior horn cells and peripheral polyneuropathy generally have asymmetric onset. 11. If there is associated fasciculations? If the patient complains of spontaneous twisting or contraction of muscles then there is associated fasciculations. It signifies LMN type of palsy. Diseases affecting the anterior horn cells or the peripheral nerves are associated with fasciculations. A patient in spinal shock will not complain of fasciculations and it is also absent in diseases acting in the neuromuscular junction or the muscles. 12. If the muscle power increases or decreases after repeated use? Patient having decrease in muscle power after repeated use will complain of maximum weakness in the evening time and will have maximum power in the morning time. Generally myasthenia gravis shows this type of character. Patient having increase in muscle power after repeated use will complain of maximum weakness in the morning time and minimum weakness in the evening. Generally Lambert-Eaton myasthenic syndrome shows this type of character. CLINICAL EXAMINATION: General examination: Decubitus- The patient will lie supine, the lower limbs will be externally rotated and there will be foot drop. The upper limbs will be in a supinated position. If the patient is unconscious there might be dribbling of saliva wetting of the clothes and the patient will not response to external noxious stimulus.

8 Nutrition- If the disease is a chronic one the nutrition will generally be poor but in acute conditions the nutrition is normal.

Appearance The patient will have a masked face if the facial muscles are involved otherwise there will be an anxious look. The accessory muscles of respiration will be prominent (Sternocleidomastoid, dilator ala nasi) if there is diaphragmatic weakness.

There will be disuse wasting of the small muscles of the hand with loss of thenar and hypothenar eminence. The foot will show disuse wasting of the small muscles and there will be pes cavus.

The upper limb and lower limb musculature will show disuse wasting which can be proximal or distal type depending upon the etiology and will be hypoplastic if the disease has occurred during the growth phase. But there can be hypertrophy in Dunchenne or Becker type of pseudohypertrophic muscular dystrophy.

There can be central type of cyanosis if the respiratory muscle involvement is severe. Anemia will be present due to malnutrition.

There will be tacypnoea with shallow respiration in diaphragmatic paresis. There will be tacycardia to compensate for hypoxia due to anemia and diaphragmatic weakness.

Nervous system: Higher mental function: They should be normal to asses the other system. The different points for evaluation of higher mental functions are o Consciousness. o Orientation. o Memory. o Intelligence. Cranial nerves:

9 o The motor cranial nerves e.g. III, IV, V, VI, VII, IX, X, XI and XII are to be examined thoroughly to look for involvement. o The other cranial nerves are to be examined for involvement. Motor system: o Bulk of the muscle The bulk is reduced due to disuse atrophy. o Tone of the muscles There will be hypotonia or flaccidity of the muscles of the four limbs. o Power of the muscle There will be decrease to complete absence of muscle power depending upon the degree of weakness. o Abnormal movements of the muscles Fasciculations are seen or can be induced if it is lower motor neuron type of palsy. The fasciculations are prominent in anterior horn cell disease and in peripheral neuropathy. It is absent in UMNP in shock state. Sensory system: o The posterior column- Involvement of predominantly the posterior column and to some extent the antero-lateral system signifies the lesion is in the peripheral nerves. If the disease is in the anterior horn cells, neuromuscular junction or in the muscles there should be no sensory involvement. o Co-ordination test- It cannot be tested if the muscle power is 3 or less. But if the power is more than 3 inco-ordination may be detected if the sensory system is involved. o Deep tendon reflex They are normal to decreases in anterior horn cell disease, neuromuscular junctional disease and myopathies but is absent in peripheral neuropathy. o Gait A patient with predominant distal weakness have high stepping gait due to foot drop. A patient with predominant proximal weakness have Waddling gait.

10 A patient with peripheral neuropathy have stumping gait.

Cerebellum: Cerebellar functions should be intact. Respiratory system: The system is to be examined to look for involvement of the respiratory muscles. A single breath count test of less than normal signifies respiratory involvement.

The cardiovascular system and abdominal system is also to be examined.

Investigations: Routine blood examination. Fasting and post-prandial blood sugar estimation. Cerebro-spinal fluid examination. CT scan of the brain and spinal cord. MRI of the brain and spinal cord. X ray of the cervical vertebrae. Electromyelography. Muscle biopsy. Nerve biopsy.

Conclusion: Cases of flaccid quadriplegia of LMN type encountered in the OPD or casualty is less. References: 1. Harrisons principles of internal medicine 12th edition. 2. Davidsons principles and practice of Medicine 19th edition.

11 3. Brains disease of the nervous system 11 edition. 4. Adams and Victors Principles of Neurology, 7th edition. 5. Guytons Textbook of medical physiology 8th edition. 6. Ganongs Review of medical physiology 12th edition. 7. Hutchinsons clinical methods 21st edition. 8. Macleods Clinical Examination 10th edition.
th

Quadriplegia (Yes / No)

Conscious UMNP LMNP

Unconscious

Ant. Horn Cell

Ventral Root

Peripheral

Neuromuscular

Muscle

or Spinal nerve

Nerve

junction