Quiz 25 on Reflexes Theme and Discussion preparation completed on 31-08-2009

1. Reflex QuizTheme & Discussion Dr. Aswini Kumar. MD Professor of Medicine Medical College Hospital Thiruvananthapuram

2. 1. Normal reflex pattern . Additional Points: Patient Conscious Higher Functions N Cranial Nerves Normal Tone Normal No Motor Deficits No Sensory Deficits No Cerebellar signs No Meningeal Signs Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator ++ Supinator ++ + Cremasteric + Knee +++ Knee +++ Ankle + Ankle + Plantar  Plantar 01

3. Reflexes Superficial and deep tendon reflexes play an important role in arriving at a neurological diagnosis and localization Superficial reflexes are designated as present (+) or absent (-). They have perhaps more important in localization Deep tendon reflexes are either Absent (-) present but sluggish (+), present normally (++) present and brisk (+++) present in an exaggerated form (++++) Exaggerated and with clonus (+++++)

4. 2. Right HemiplegiaNeuronal Shock . Additional Points: Patient Conscious Motor Aphasia UMN Facial [Right] Hypotonia [Right] Grade 0 power [Right] No Sensory Deficits No Cerebellar signs No Meningeal Signs Conjval + Corneal + Jaw - Triceps ++ Triceps- Biceps++ Biceps - Abdominal - + - + Spinatore ++ Supinator - - Cremasteric + Knee +++ Knee - Ankle + Ankle - Plantar  Plantar 02

5. Hemiplegia Hemiplegia is a condition in which upper limb and lower limb on one side of a patient's body is paralyzed It is usually the result of a stroke, although other diseases affecting the hemispheres are also capable of producing it Rarely it can follow processes affecting the brainstem (crossed hemiplegia) and spinal cord (without VII palsy) It's a pyramidal tract lesion from the origin at the cerebral cortex down to 5th cervical segment of the spinal cord During the initial few days there is loss of all neuronal functions – Neuronal shock state- important in prognosis

6. 3. Left HemiplegiaPost shock . Additional Points: Patient Conscious Higher Functions N Left UMN Facial Clasp Knife [Left] Grade 2 [Left] No Sensory Deficits No Cerebellar signs No Meningeal Signs Conjval + Corneal + Jaw - Triceps +++ Triceps++ Biceps +++ Biceps ++ Abdominal + - + - Spinatore +++ Supinator ++ + Cremasteric - Knee ++++ Knee +++ Ankle ++ Ankle + Plantar  Plantar 03

7. Causes of Hemiplegia 1-vascular:- stroke ((the most common cause) 2-infective:- encephalitis -meningitis - brain abscess 3-neoplastic:- glioma-meningioma 4-demylination:- DS disseminated sclerosis 5-traumatic:- cerebral lacerations 6-congenital:-cerebral palsy 7-hysterical:-absence of organic pyramidal lesion 8-Post-ictal :- Todd’s paralysis 9-Migraine :- Hemiplegic migraine

8. 4. Old Right Hemiplegia . Additional Points: Patient Conscious UMN Facial [Right] Tone [Right] Contractures [Right] Power Grade 3 [Right] Patellar Clonus [Right] Ankle Clonus [Right] RHD MS Atrial Fib Conjval + Corneal + Jaw - Triceps ++ Triceps++++ Biceps ++ Biceps ++++ Abdominal - + - + Spinatore ++ Supinator +++ - Cremasteric + Knee +++ Knee ++++ Ankle + Ankle +++ Plantar  Plantar 04

9. Causes of Embolic Stroke Aorta:- Atheromatous Plaques (Artery to Artery Embolism) Aortic Root:- Aortic Aneurysm with thrombus, Aortitis Aortic Valve:- Endocarditis, Prosthetic Valve Thrombus Left Ventricle:- Mural Thrombus from Post MI dyskinesia Mitral Valve:- Stenosis, Endocardittis, MVR with thrombus Left Atrium:- Thrombus in MS or AF or both , Myxoma, Pulmonary Veins:- Pulmonary Venous Thrombosis, Lung Parenchyma,: Lung Abscess, Bronchiectasis, Tumors Emboli passing through lungs:- Fat , Air, Amniotic Fluid Paradoxical Embolism:- Fallot’sEisenmenger Syndrome

10. 5. Pseudo-bulbarPalsy . Conjval + Corneal + Jaw +++ Triceps +++ Triceps+++ Biceps +++ Biceps +++ Abdominal - - - - Spinatore +++ Supinator +++ - Cremasteric - Knee ++++ Knee ++++ Ankle ++ Ankle++ Plantar Plantar 05 Additional Points: Patient Conscious Emotional Incontinence Exaggerated Gag reflex Hypertonia B/L Variable weakness Primitive Reflexes + No Cerebellar signs No Meningeal Signs

11. Pseudo-bulbar palsy Pseudo-bulbar palsy is bilateral impairment of the function of the lower cranial nerves 9, 10, 11 and 12 which control the muscles of eating, swallowing and talking It is the result of an upper motor neuron lesion to the cortico-bulbar pathways in the pyramidal tract Causes Vascular causes: Bilateral hemisphere infarction Degenerative disorders: motor neuron disease Inflammatory disorders: Multiple sclerosis Malignancy: High brain stem tumors Brain trauma

12. 6. Parkinson’s Disease . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Spinatore ++ Supinator ++ + Cremasteric + Knee +++ Knee +++ Ankle + Ankle + Plantar  Plantar 06 Additional Points: Patient Conscious Expressionless face Cranial Nerves Normal Cog wheel rigidity Generalized Hypokinesia Glabellar Tap Resting tremor No Meningeal Signs

13. Parkinson's disease Parkinson's diseaseis a degenerative disorder of CNS that often impairs the sufferer's motor skills and speech Parkinson's disease belongs to a group of conditions called movement disorders It is characterized by muscle rigidity, tremor, slowing of physical movements, bradykinesia or akinesia The primary symptoms are the results of decreased stimulation of the motor cortex by the basal ganglia It is caused by the insufficient formation and action of dopamine, which is produced by dopaminergic neurons

14. 7. Lateral MedullarySyndrome [Left] . Conjval - Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Spinatore ++ Supinator ++ + Cremasteric + Knee +++ Knee +++ Ankle + Ankle + Plantar  Plantar 07 Additional Points: Patient Conscious Vertigo and ataxia Vomiting and hiccups Horner’s Syndrome[Left] IX & X palsy [Left] Pain & Temp lost Lt face Pain & Temp lost Rt body Cerebellar signs [Left]

15. Lateral Medullary Syndrome It is also called Wallenberg's syndrome and posterior inferior cerebellar artery syndrome) It is caused by an infarct caused by interrupted blood supply to lateral parts of the medulla Symptoms and findings are ataxia, facial pain, vertigo nystagmus, Horner‘s syndrome, diplopia and dysphagia Ipsilateral pain and temp loss over face and contralateral pain and temp over body The cause of this syndrome is usually the occlusion of the posterior inferior cerebellar artery (PICA) at its origin.

16. 8. QuadriplegiaC3 Level . Conjval + Corneal + Jaw - Triceps +++ Triceps+++ Biceps +++ Biceps +++ Abdominal - - - - Supinator ++ Supinator ++ - Cremasteric - Knee ++++ Knee ++++ Ankle +++ Ankle +++ Plantar  Plantar  08 Additional Points: Patient Conscious Respiratory distress Weakness all 4 limbs HypertoniaBilateral Distal > proximal weakness Total sensory loss below C3 Pectoral&Trapezius Jerk+++ UMN Bladder

17. Quadriplegia Also known as tetraplegia, it is paralysis caused by illness or injury to spinal cord resulting in weakness of all 4 limbs A loss or impairment in controlling bowel and bladder, sexual function, digestion, breathing, autonomic functions Sensation is usually involved in affected areas, manifesting as numbness, reduced sensation, or neuropathic pain Complications: Pressure sores, osteoporosis and fractures, frozen joints, spasticity, respiratory complications and infections, autonomic dysreflexia, deep vein thrombosis

18. 9. Right C5Radiculomyelopathy . Conjval + Corneal + Jaw - Triceps +++ Triceps+++ Biceps ++ Biceps - Abdominal - - - - Supinator ++ Supinator Inverted - Cremasteric - Knee ++++ Knee ++++ Ankle +++ Ankle +++ Plantar  Plantar  09 Additional Points: Patient Conscious Higher Functions N Cranial Nerves Normal Hypertonia Lower limbs Shoulder abduction  Elbow flexion  Sensory loss along C5 C6 UMN Bladder

19. Cervical Spondylosis It is degenerative arthritis of the joints between the centra of spinal vertebrae; the interfacetal joints are not involved When the space between two adjacent vertebrae narrows, compression of a nerve root result in radiculopathy Sensory and motor disturbances, such as severe pain in the neck, shoulder, arm, back, and/or leg, & muscle weakness Less commonly, direct pressure on the cervical spinal cord may result in clinical features of quadriparesis (myelopathy) Here there is global weakness, gait dysfunction, loss of balance, and loss of bowel and/or bladder control

20. 10.SyringomyeliaC-8,T-1 Level . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal - - - - Supinator ++ Supinator ++ - Cremasteric - Knee ++++ Knee ++++ Ankle +++ Ankle +++ Plantar  Plantar 10 Additional Points: Patient Conscious HMF and Cranial Normal Hypotoniadistally both ULs Small muscle wasting ULs Handgrip weakness B/L Motor weakness both LL Suspended sensory loss C8T1 Sensory Dissociation

21. Syringomyelia A cyst or cavity, called a syrinx forms within the spinal cord, expand and elongate over time, destroying the cord This damage may result in pain, weakness, and stiffness in the back, shoulders, and extremities The disorder generally leads to a cape-like loss of pain and temperature sensation along the back and arms Some patients experience paralysis or paresis of the lower limbs temporarily or permanently The symptoms typically vary depending on the extent and, the location of the syrinx within the spinal cord

22. 11. ParaplegiaT-10 Level . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + - - Supinator ++ Supinator ++ - Cremasteric - Knee ++++ Knee ++++ Ankle +++ Ankle +++ Plantar  Plantar 11 Additional Points: Patient Conscious Higher Functions N Cranial Nerves Normal Hypertonia both LLs Grade 0 both LLs Beevor’s sign + Sensory level at Umbilicus UMN Bladder

23. Acute Transverse Myelitis Neurological disorder caused by an inflammatory process of the spinal cord, which can result in axonal demyelination This demyelination arises idiopathically following infection, vaccination or multiple sclerosis Symptoms include weakness and numbness of the limbs as well as motor, sensory, and sphincter deficits A major differentiation or distinction to be made is a compression of the spinal cord in the spinal canal Treatment is usually symptomatic only, corticosteroids being used with limited success

24. 18.Right T11 Brown- Sequard . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + - + Supinator ++ Supinator ++ - Cremasteric + Knee +++ Knee - Ankle + Ankle- Plantar  Plantar 12 Additional Points: Patient Conscious Higher Functions N Right lower limb grade 2 Tone reduced right LL Pain and temp lost left LL Vibration and joint position lost right LL Right T11 hyperaesthesia

25. Brown- Sequard Syndrome Loss of sensation and motor function (paralysis and ataxia) that is caused by the lateral hemi-section of the spinal cord Features of a motor loss on the same side of spinal injury and loss of pain and temperature sensation on opposite side Caused by a spinal cord tumor or trauma (such as a gunshot wound or puncture wound to the neck or back), Other causes: ischemia (obstruction of a blood vessel), or infectious or inflammatory diseases such as tuberculosis The syndrome was first described in 1850 by the British / Mauritian neurologist Charles-Édouard Brown Séquard

26. 13. ParaplegiaL-1 Level - Shock . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator ++ Supinator ++ -Cremasteric - Knee - Knee - Ankle - Ankle - Plantar  Plantar 13 Additional Points: Patient Conscious Higher Functions N Cranial Nerves Normal Hypotonia LLs Power grade 0 both LL Total sensory loss below L1 No Cerebellar signs UMN Bladder

27. Flaccid Paraplegia The area of the spinal canal which is affected in paraplegia is either the thoracic, lumbar, or sacral regions. The causes range from trauma (acute spinal cord injury: transsection or compression of the cord, vertebral fractures The most common cause of paraplegis (and all spinal cord injuries) is motor vehicle accidents Other causes include violence, sports, cancer involving the epidural or dural space, vertebral fractures and myelitis Sometimes paralysis of both legs can result from injury to the brain, or tumors like a para-sagitalmeningioma

28. 14. CaudaEquinaSyndrome . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator++ Supinator ++ + Cremasteric + Knee+ Knee - Ankle - Ankle - Plantar - Plantar- 14 Additional Points: Patient Conscious Higher Functions N Asymmetric Wasting of LL Hypotonia both LL Weakness below L3 Patchy sensory loss below L3 No Cerebellar signs Late Bladder involvement

29. Caudaequina syndrome is a condition in which there is acute loss of function of the nerve roots of the spinal canal below the termination conus Caudaequina contains the nerve roots from L1-5 and S1-5. The nerve roots from L4-S4 join in the sacral plexus Signs: weakness of the muscles innervated by the compressed roots, often an asymetrical paraplegia Sphincter weaknesses causing urinary retention decreased anal tone; sexual dysfunction & saddle anesthesia Bilateral leg pain and weakness; and bilateral absence of ankle reflexes. Pain and weakness may, however, be absent

30. 15.Amyotrophic Lateral Sclerosis . Conjval + Corneal + Jaw ++ Triceps +++ Triceps+++ Biceps +++ Biceps +++ Abdominal + + + + Spinator +++ Supinator +++ + Cremasteric + Knee ++++ Knee ++++ Ankle +++ Ankle +++ Plantar  Plantar  15 Additional Points: Patient Conscious Higher Functions N Wasting hands & calf Tone Increased B/L Variable Weakness No Sensory Deficits Fasciculations present No bladder involvement

31. Amyotrophic lateral sclerosis Amyotrophic lateral sclerosis is a form of motor neuron disease. ALS, sometimes is called Maladie de Charcot It is a progressive, fatal, neurodegenerative disease caused by the degeneration of motor neurons The disorder causes muscle weakness and atrophy throughout the body as both the UMN & LMN degenerate The muscles gradually weaken, develop fasciculations because of denervation, and eventually atrophy Cognitive function is generally spared except when ALS is associated with frontotemporal dementia

32. 16. GullianBarreSyndrome . Conjval + Corneal + Jaw - Triceps - Triceps- Biceps - Biceps - Abdominal - - - - Supinator- Supinator - -Cremasteric - Knee - Knee - Ankle- Ankle - Plantar -- Plantar -- 16 Additional Points: Patient Conscious Higher Functions N Acute Ascending paralysis Markedly reduced tone No Sensory Deficits Single Breath Count < 20 Bilateral LMN Facial No Bladder involvement

33. GullianBarre Syndrome An acute inflammatory demyelinating polyneuropathy, also an autoimmune disorder affecting peripheral nervous system It is frequently severe and usually exhibits as an ascending paralysis in the legs that spreads to the upper limbs The face may be affected in a bilateral upper motor neuron paralysis along with complete loss of deep tendon reflexes. With plasmapheresis or IVIg and supportive care, the majority of patients will regain full functional capacity However, death may occur if severe pulmonary complications arise and dysautonomia are present

34. 16.Peripheral Neuropathy . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator - Supinator - + Cremasteric + Knee+ Knee+ Ankle - Ankle - Plantar / Plantar/ 17 Additional Points: Patient Conscious Higher Functions N Cranial Nerves Normal Tone Normal Distal weakness UL & LL Glove & Stocking sensory No Cerebellar signs No Meningeal Signs

35. Peripheral neuropathy It is the term for damage to nerves of the peripheral nervous system, either by diseases of the nerve or The causes are broadly grouped as follows: Genetic diseases: Charcot-Marie-Tooth syndrome Metabolic/Endocrine: diabetes, porphyria, amyloidosis Toxic causes: alcoholism, phenytoin, isoniazid, metals Inflammatory diseases: GBS, SLE, Leprosy, Sjögren's Vitamin deficiency states: Vitamin B12, Vitamin B1 Others: Malignant disease, HIV, radiation, chemotherapy

36. 18. MyastheniaGravis . Additional Points: Patient Conscious Higher Functions N Bilateral Partial Ptosis Tone Normal Fatigability No Sensory Deficits No Cerebellar signs No Meningeal Signs Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator++ Supinator ++ + Cremasteric + Knee +++ Knee +++ Ankle + Ankle + Plantar  Plantar 18

37. Myasthenia gravis It is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability It is anautoimmune disorder, in which weakness is caused by circulating antibodies These block acetylcholine receptors at the post-synaptic neuromuscular junction Thus inhibiting the stimulative effect of the neurotransmitter acetylcholine. Myasthenia is treated with cholinesterase inhibitors or immunosuppressants and in selected cases, thymectomy

38. 19. Friedrichsataxia . Conjval + Corneal + Jaw - Triceps + Triceps+ Biceps + Biceps + Abdominal + + + + Spinatore - Supinator - + Cremasteric + Knee ++ Knee ++ Ankle - Ankle - Plantar Plantar 19 Additional Points: Symptomatic before 20 years Higher Functions N Pescavus, kyphoscoliosis H/o ataxia in family Power grade 4 all limbs Ataxia Vibration, joint position impaired distally

39. Friedrichs ataxia An inherited disease that causes progressive damage to the nervous system It results in symptoms ranging from gait disturbance and speech problems to heart disease Ataxia results from degeneration of nerve tissue in spinal cord, in particular sensory neurons connection with cerebellum The condition is named after the German physician NicholausFriedreich, who first described it in the 1860s is caused by a mutation in gene FXN (formerly known as X25) that codes for frataxin, located on chromosome 9

40. . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Spinatore - Supinator - + Cremasteric + Knee +++ Knee +++ Ankle - Ankle - Plantar Plantar 20 Additional Points: Patient Conscious Higher Functions irritable Optic atrophy Grade 4 power all 4 limbs Loss of vibration and joint position sense Megaloblastic anemia in peripheral smear 20. Subacute combined degeneration of spinal cord

41. Subacute combined degeneration of spinal cord Degeneration of the posterior and lateral columns of the spinal cord as a result of vitamin B12 deficiency Also known as Lichtheim's disease, it is usually associated with pernicious anemia. Patients present with weakness of legs , arms, trunk, tingling and numbness that progressively worsens Bilateral spastic paresis may develop and pressure, vibration and touch sense are diminished Therapy with vitamin B12 results in partial to full recovery, depending on the duration and extent of neuro-degeneration

42. 21.Facioscapulo humeral dystrophy . Conjval + Corneal + Jaw - Triceps - Triceps- Biceps - Biceps - Abdominal + + + + Spinatore + Supinator+ + Cremasteric + Knee + Knee + Ankle + Ankle + Plantar  Plantar 21 Additional Points: Facial weakness, shoulder girdle weakness,winging of scapula,deltoid preserved, pseudohypertrophy present Tone reduced Wrist extensors grade 2, flexors grade 3 No Sensory Deficits No Cerebellar signs No Meningeal Signs

43. Facioscapulo humeral dystrophy Also known as Landouzy-Dejerine Disease, it is an autosomal dominant form of muscular dystrophy It initially affects the skeletal muscles of the face (facio), scapula(scapulo) and upper arms (humeral) Symptoms may develop in early childhood and are usually noticeable in the teenage years with 95% by age 20 years A progressive skeletal muscle weakness usually develops in areas of the body; often the weakness is asymmetrical Non-muscular symptoms associated with FSHD include subclinical sensorineural hearing loss & retinal telangectasias

44. 22. Cerebral palsy . Additional Points: H/O hypoxia at the time of birth Non progressive weakness of LL more than UL Tone Increased Scissoring gait No Sensory Deficits Conjval + Corneal + Jaw+ Triceps +++ Triceps+++ Biceps +++ Biceps +++ Abdominal - - - - Spinatore+ ++ Supinator+ ++ - Cremasteric - Knee ++++ Knee ++++ Ankle ++ Ankle ++ Plantar Plantar 22

45. Cerebral palsy Non-progressive motor,non-contagious conditions that cause physical disability in human development Caused by damage to the motor control centers of the developing brain and can occur during pregnancy (75%) Can also occur during childbirth (5%) or after birth (about 15 %) up to about age three Accompanied by disturbances of sensation, perception cognition, communication, and behaviour, & by epilepsy There is no known cure for CP. Medical intervention is limited to the treatment and prevention of complications

46. 23. CIDP . Additional Points: Chronic history Relapsing remitting Patient Conscious Higher Functions N Proximal and distal weakness Tone reduced Sensory loss distally Conjval + Corneal + Jaw - Triceps - Triceps- Biceps - Biceps - Abdominal - - - - Spinatore - Supinator - -Cremasteric - Knee - Knee - Ankle- Ankle - Plantar  Plantar 23

47. Chronic inflammatory demyelinating polyneuropathy It is an acquired immune-mediated inflammatory disorder of the peripheral nervous system but may be CNS involvement Closely related to Guillain-Barré syndrome and it is considered the chronic counterpart of that acute disease The pathologic hallmark is loss of the myelin sheath (the fatty covering that protects nerve fibers) of peripheral nerves Numbing, tingling, pain, progressive muscle weakness, loss of deep tendon reflexes, fatigue, and abnormal sensations Though conventional electrophysiological diagnostic criteria are not met, patient may still respond to immunomodulation

48. 24. Anterior spinal artery occlusion . Conjval + Corneal + Jaw - Triceps ++ Triceps++ Biceps ++ Biceps ++ Abdominal + + + + Supinator ++ Supinator ++ -Cremasteric - Knee - Knee - Ankle - Ankle - Plantar  Plantar 24 Additional Points: Hyper acute onset Patient Conscious Higher Functions N Hypotonia both LL Grade 0 power LL Pain and temperature lost both LL Vibration and joint position preserved

49. Anterior spinal artery occlusion Ischemia or infarction of the spinal cord in the distribution of the anterior spinal artery, which supplies the ventral 2/3 Causes: Atherosclerosis of the aorta; dissection of Aortic aneurysm; dissection of the anterior spinal artery or Arteritis Clinical features include weakness and loss of pain and temperature sensation below the level of injury to cord It is an abrupt onset flaccid paralysis of the legs with relative sparing of position and vibratory sensation It is also known as "Beck's syndrome“, Sphincter control and sexual disorders are usually present

50. 25. Myotonic dystrophy . Conjval + Corneal + Jaw - Triceps+- Triceps+ Biceps + Biceps + Abdominal + + + + Supinator+ Supinator+ + Cremasteric + Knee + Knee + Ankle + Ankle + Plantar  Plantar 25 Additional Points: Patient Conscious Frontal balding Bilateral Partial Ptosis Hatchet facies Testicular atrophy Grade 4 power proximally and grade 2 distally Myotonia No Sensory Deficits

51. Myotonic dystrophy Dystrophiamyotonica, is a chronic, slowly progressing, highly variable inherited multi-systemic disease It is characterized by wasting of the muscles (muscular dystrophy), and myotonia (difficulty relaxing a muscle) Posterior subcapsular iridescent cataracts (opacity of the lens of the eyes), conduction defects & endocrine changes DM1 also known as Steinert's disease is a congenital form; DM2 also as PROMM or proximal myotonicmyopathy Most notably, the disease shows at an earlier age in successive generations, a phenomenon termed anticipation

52. Thank You