Neurogenic Conditions


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Neurogenic Conditions

  1. 1. Neurogenic Conditions
  2. 2. Fourth Nerve (or Superior Oblique) Palsy <ul><li>Common as both congenital and acquired </li></ul><ul><li>Can be bilateral or unilateral </li></ul>
  3. 3. Aetiology <ul><li>Familial defect of nerve or its nucleus, or major brain abnormality </li></ul><ul><li>Many unknown </li></ul><ul><li>Head injury, eg damage to trochlea in upper cut blow in boxing </li></ul><ul><li>Diabetes </li></ul><ul><li>Intracranial tumour </li></ul>
  4. 4. Characteristics <ul><li>Bielschowsky Head Tilt Test : this differentiates SO palsy from the contralateral SR Palsy </li></ul>
  5. 6. Ocular Posture <ul><li>Unilateral palsies: </li></ul><ul><li>affected eye hypertropic and slightly esotropic </li></ul><ul><li>Excyclotropia likely </li></ul><ul><ul><li>Hypertropia increases on near fixation </li></ul></ul><ul><ul><li>Slight V pattern </li></ul></ul><ul><li>Bilateral palsies: </li></ul><ul><ul><li>Constant V pattern esotropia with hypertropia of the non-fixing eye </li></ul></ul><ul><ul><li>Extorsion of non-fixing eye in acquired only </li></ul></ul>
  6. 7. AHP <ul><li>Unilateral: </li></ul><ul><li>(I) Head tilt to sound side </li></ul><ul><li>(ii) Face turn to sound side </li></ul><ul><li>(iii) Chin Depression </li></ul><ul><li>All are to overcome the vertical diplopia </li></ul><ul><li>Bilateral: Chin depression </li></ul>
  7. 8. Hess Chart <ul><li>Field of BSV is displaced upwards (and to affected side in unilateral) </li></ul>
  8. 9. Management <ul><li>Acquired unilateral palsy = vertical prism </li></ul><ul><li>Others = surgery </li></ul><ul><li>90% of isolated unilateral palsies (except some traumatic ones) recover spontaneously within 6 months. </li></ul>
  9. 10. Sixth Nerve (Lateral Rectus) Palsy <ul><li>Common </li></ul><ul><li>Aetiology </li></ul><ul><ul><li>Birth trauma: instrumentation, increases intracranial pressure presses nerve against the petrous temporal bone </li></ul></ul><ul><ul><li>Childhood: meningitis, otitis media, pneumonia etc </li></ul></ul><ul><ul><li>Adult:trauma, demyelinating disease, intracranial space occupying lesions </li></ul></ul><ul><ul><li>Old Age: CVA </li></ul></ul>
  10. 11. Characteristics <ul><li>Ocular Posture esotropia on distance fixation with less for near (or only phoria at near) </li></ul><ul><li>Crossed fixation and equal VA in congenital bilateral type </li></ul>
  11. 12. AHP <ul><li>Unilateral = face turn to affected side (esp in distance fixation) </li></ul>
  12. 13. Hess Chart <ul><li>Field of BSV displaced to unaffected side in unilateral </li></ul>
  13. 14. Management <ul><li>Prism base out to distance portion of bifocals </li></ul><ul><li>Botulinium toxin A injection into MR reduces esotropia to alleviate diplopia and prevents contracture of ipsilateral MR </li></ul><ul><li>Occlusion to prevent amblyopia in children </li></ul><ul><li>Surgery </li></ul>
  14. 15. Related Syndromes to 6 th Nerve Palsy <ul><li>Mobius’ syndrome </li></ul><ul><li>Congenital. </li></ul><ul><li>Due to close association of 6 th and 7 th cranial nerves in the mid-brain </li></ul><ul><li>This syndrome is bilateral 6 th and 7 th nerve palsies, causing loss of abduction and facial weakness (not necessarily symmetrical) </li></ul><ul><li>Expressionless face, incomplete lid closure, bilateral loss of abduction etc </li></ul>
  15. 16. Graderigos Syndrome <ul><li>LR palsy caused by a middle ear infection which spreads to the petrous bone causing swelling and affecting the 5 th (Trigeminal) and 6 th nerves </li></ul><ul><li>Results in 6 th nerve palsy, ipsilateral trigeminal pain of the face, deafness and facial turn to the affected side </li></ul><ul><li>This condition is rare since the advent of antibiotics </li></ul>
  16. 17. 3 rd Nerve Palsy (Ophthalmoplegia) <ul><li>Mostly unilateral </li></ul><ul><li>Possible causes: Birth injury, head injury, diabetes, hypertension, compression by posterior communicating artery aneurysm </li></ul>
  17. 18. Classification <ul><li>Complete Oculomotor Palsy affects all extra- and intra- ocular muscles. </li></ul><ul><li>Divergent strabismus with slight depression, </li></ul><ul><li>ptosis, </li></ul><ul><li>mydriasis, </li></ul><ul><li>loss of pupil action </li></ul><ul><li>accommodation </li></ul>
  18. 19. Partial Paresis <ul><li>External ophthalmoplegia (all external ocular muscles only) </li></ul><ul><li>Internal ophthalmoplegia (paresis of ciliary muscle and iris sphincter) </li></ul><ul><li>Superior division of 3 rd Nerve (SR and levator) </li></ul><ul><li>Inferior division (IR, MR, IO, ciliary muscle and sphincter </li></ul><ul><li>Double elevator palsy (SR and IO). Congenital </li></ul>
  19. 20. Single Muscle Paresis (usually congenital) <ul><li>SR with V pattern exotropia (usually accompanied by ptosis) </li></ul><ul><li>MR (very rare) </li></ul><ul><li>IR (least common) </li></ul>
  20. 21. Cyclic Oculomotor Palsy <ul><li>intermittant 3 rd Nerve Palsy. </li></ul><ul><li>Rare, </li></ul><ul><li>? Cause </li></ul><ul><li>An acquired SR palsy is generally accompanied by ptosis since the SR and levator share the superior division of the 3 rd nerve and are anatomically in close proximity </li></ul><ul><li>Often get accompanying symptoms eg HIA, a tremor of the contalateral limbs (due to involvement of the Red Nucleus = Benedikts’ syndrome, and other diabetic symptoms). </li></ul>
  21. 22. Management <ul><li>Prisms – limited use (diplopia only present if incomplete ptosis) </li></ul><ul><li>Botulinum toxin A – injection into LR can be helpful </li></ul><ul><li>Pilocarpine 0.1% to reduce photophobia </li></ul><ul><li>Surgery </li></ul>
  22. 23. Mechanical Restriction of Ocular Movement
  23. 24. Duane’s Retraction Syndrome <ul><li>Congenital </li></ul><ul><li>Bilateral or unilateral – more often affecting LE </li></ul><ul><li>Incidence higher in females </li></ul>
  24. 25. Characteristics <ul><li>One or all of: </li></ul><ul><li>Limitation of abduction with widening of palpebral aperture </li></ul><ul><li>Less marked limitation of adduction in the same eye </li></ul><ul><li>Retraction of globe on adduction with narrowing of palpebral apertures </li></ul><ul><li>Poor convergence </li></ul><ul><li>Face turn to affected side </li></ul>
  25. 26. Classification <ul><li>TYPE A:Limited abduction, less marked limitation of adduction </li></ul><ul><li>TYPE B:Limited abduction but normal adduction </li></ul><ul><li>TYPE C:Limitation of adduction exceeds limitation of abduction. Exotropia and face turn </li></ul>
  26. 27. Aetiology <ul><li>Innervation of the LR by extra branches of the 3 rd nerve in place of an absent or atrophic 6 th nerve </li></ul><ul><li>Simultaneous contraction of MR and LR resulting in retraction </li></ul><ul><li>Lack of abduction because lack of 6 th nerve innervation </li></ul><ul><li>Fibrosis of LR results, and hence mechanical restriction </li></ul>
  27. 28. Management <ul><li>Treatment of amblyopia and correction of refractive error </li></ul><ul><li>Surgery for cosmetic correction of AHP, if necessary. </li></ul>
  28. 29. Starbismus Fixus <ul><li>Characteristics </li></ul><ul><li>Very marked Esotropia </li></ul><ul><li>Fibrosis and contracture of both MR – hence adduction of both eyes </li></ul><ul><li>Absence of active and passive horizontal eye movement </li></ul><ul><li>Face turn to side of fixing eye </li></ul><ul><li>Rare </li></ul><ul><li>Presents in infancy </li></ul><ul><li>Uncertain if fibrosis of MR is a primary condition or secondary to LR paralysis </li></ul>
  29. 30. Adherence Syndromes <ul><li>Either: congenital adherence of extra-ocular muscles to each other or to facial tissue or levator Familial </li></ul><ul><li>Or: acquired due to infection, trauma or more surgery (especially strabismus or retinal detachment surgery) </li></ul><ul><li>Management; Surgery </li></ul>
  30. 31. Brown’s Syndrome (or Superior Oblique Tendon Sheath Syndrome) <ul><li>Develops in infancy, or acquired later through inflammation (eg rheumatoid arthritis) or trauma in the trochlear region. </li></ul><ul><li>Mainly unilateral, can be bilateral </li></ul>
  31. 32. Characteristics <ul><li>Limitation of elevation in adduction </li></ul><ul><li>Overaction of the contralateral SR but other muscle sequelae do not develop (therefore no defect in lower field) </li></ul><ul><li>A or V pattern </li></ul><ul><li>Typical Hess chart </li></ul><ul><li>In some acquired cases with repeated attempts to look up and in an audible ‘click’ is heard and eye suddenly shoots up with overacting IO </li></ul><ul><li>AHP: tilt to affected side (Not always present) </li></ul><ul><li>Positive forced duction test (Negative in IO palsy) </li></ul>
  32. 33. Aetiology <ul><li>Uncertain and variable. </li></ul><ul><li>Main causes: Short tendon sheath </li></ul><ul><li>Presence of swelling on SO Tendon preventing free passage through trochlea </li></ul>
  33. 34. Management <ul><li>Most spontaneously recover </li></ul><ul><li>Some surgery for cosmetic correction of AHP </li></ul>
  34. 35. Blow-Out Fracture <ul><li>Caused by direct blow to eye through closed lids </li></ul><ul><li>Wave of pressure causes orbital floor to fracture, and a portion of orbital tissue may become trapped </li></ul><ul><li>This restricts rotation of the globe, particularly up and down </li></ul>
  35. 36. Management <ul><li>May recover spontaneously within 2 weeks </li></ul><ul><li>Others require surgery to free trapped tissue </li></ul>
  36. 37. Myogenic Palsies <ul><li>One in which the weakness of ocular movement is due to a primary problem affecting the muscle itself rather one disrupting the nerve supply or causing mechanical restriction. </li></ul>
  37. 38. Myasthenia Gravis <ul><li>Comparatively rare disorder of neuromuscular transmission </li></ul><ul><li>Onset 20-40 years. </li></ul><ul><li>Characterised by excessive fatiguability of striated muscle, most commonly the EOMs but may be more generalised. </li></ul><ul><li>Diplopia and Ptosis are often presenting symptoms, which worsen during the day. </li></ul><ul><li>May later progress to a more generalised muscle involvement. </li></ul><ul><li>The condition generally stabilises and then help may be gained with prisms, ptosis props or surgery. </li></ul><ul><li>Can get ptosis and can be confused with 3 rd nerve palsy </li></ul>
  38. 39. Orbital Myositis <ul><li>Inflammatory swelling of one or more of the EOMs. </li></ul><ul><li>Self limiting and resolves in about 8 weeks </li></ul>
  39. 40. Rhabdomyosarcoma <ul><li>Highly malignant tumour of the striated muscle </li></ul><ul><li>May produce a strabismus and restriction of ocular movement or acute proptosis </li></ul><ul><li>Usually in childhood </li></ul>
  40. 41. Dysthyroid Eye Disease (Ophthalmic Grave’s Disease) <ul><li>Can occur in hyperthyroidism (thyrotoxicosis) </li></ul><ul><li>Or hypothyroidism (myxoedema) </li></ul><ul><li>But is most common in the former </li></ul><ul><li>Can present as a muscle palsy without exophthalmus </li></ul><ul><li>Limitation of elevation, but more than one muscle is affected, in the following sequence </li></ul><ul><li>swelling of EOM (wet phase) = MYOGENIC </li></ul><ul><li>fibrosis and secondary muscle contracture, especially of the IR (dry phase) = RESTRICTIVE </li></ul>
  41. 42. Dysthyroid Eye Disease (Ophthalmic Grave’s Disease) <ul><li>Other eye signs: proptosis, von Graefe’s sign, lid retraction, decreased blinking chemosis and vertical diplopia </li></ul><ul><li>Other symptoms may be: </li></ul><ul><ul><li>increased/decreased appetite (depends on whether hyper/hypo) </li></ul></ul><ul><ul><li>decrease/increase weight </li></ul></ul><ul><ul><li>clammy hands </li></ul></ul><ul><ul><li>increased/decreased heart rate </li></ul></ul><ul><ul><li>anxiety/lethargy </li></ul></ul><ul><ul><li>Tremor in hyper </li></ul></ul>
  42. 43. Other systemic conditions causing ocular palsies <ul><li>Diabetes </li></ul><ul><li>Disease of the small blood vessel network (vaso nervorum) </li></ul><ul><li>May cause acquired 3 rd , 4 th or 6 th Nerve palsy (Third most common) </li></ul><ul><li>Usually spares the pupil (these fibres lie superficially in Third Nerve and are not supplied by small vessel network as much) </li></ul><ul><li>Diplopia </li></ul>
  43. 44. Other symptoms: severe headache, increased thirst, increased urination, increased appetite, decreased weight, constipation, boils and skin conditions. More common in older overweight females. Diabetes <ul><li>Other symptoms: </li></ul><ul><ul><li>severe headache, </li></ul></ul><ul><ul><li>increased thirst, </li></ul></ul><ul><ul><li>increased urination, </li></ul></ul><ul><ul><li>increased appetite, </li></ul></ul><ul><ul><li>decreased weight, </li></ul></ul><ul><ul><li>constipation, </li></ul></ul><ul><ul><li>boils and skin conditions. </li></ul></ul><ul><ul><li>More common in older overweight females. </li></ul></ul>
  44. 45. Hypertension <ul><li>Most commonly affects the 6 th nerve, mostly in old age </li></ul><ul><li>Symptoms: </li></ul><ul><ul><li>Headache, </li></ul></ul><ul><ul><li>dizziness, </li></ul></ul><ul><ul><li>breathlessness, </li></ul></ul><ul><ul><li>tinitis </li></ul></ul>
  45. 46. Temporal arteritis (Giant Cell Arteritis) <ul><li>Inflammatory disease of temporal artery occurring in over 60s </li></ul><ul><li>Rarely presents as isolated oculomotor nerve palsy </li></ul><ul><li>Symptoms: </li></ul><ul><ul><li>scalp tenderness, </li></ul></ul><ul><ul><li>headache, </li></ul></ul><ul><ul><li>prominent temporal arteries, </li></ul></ul><ul><ul><li>general malaise </li></ul></ul><ul><ul><li>weight loss </li></ul></ul>
  46. 47. Multiple Sclerosis <ul><li>Ocular palsy is an early symptom in many patients </li></ul><ul><li>Other symptoms: loss of muscle coordination, limb weakness, slurring of speech </li></ul>