A 70 years old man with Bilateral Upper limbs weakness and Swallowing difficulty

A 70 years old man with Bilateral Upper limbs weakness and Swallowing difficulty

1. Welcome

2. A 70 years old man with Bilateral Upper limbs weakness and Swallowing difficulty Dr. Md. Mohiminul islam Fahim Assistant Registrar Medicine Unit 3 BSMMCH, Faridpur

3. Particulars of the patient NAME : Prosanto Kumar Shaha AGE : 70 Years Sex : Male Religion : Hindu Marital status : Married OCCUPATION : Business ADDRESS : Madhukhali , Faridpur DATE OF AD M ISSION : 11. 07 .24 DATE OF EXAMINATION : 11. 07. 24

4. CHIEF COMPLAINTS Weakness of both upper limbs for 1 year Difficulties in swallowing for same duration Weight loss for same duration

5. According to the patient’s statement he was reasonably well 1 year back. Then he developed weakness in both upper limbs. He felt it during weight lifting and hair combing . W eakness i s gradual in onset & progressive in nature , associated with muscle wasting. The weakness was symmetrical and initially affecting the distal muscle then proximal muscle. There i s no association of any abnormal sensation, diurnal variation & easy fatiguability. History of Presenting Illness

6. He also noticed difficulty in initiation of swallowing which occurs both in liquid and solid foods with some nasal regurgitation. Cont …

7. The patient gave history of weight loss which is not related with excessive sweating thirst, polyuria, cough or diarrhoea , He also experienced occasional twitching movement in different parts of the body. He has no previous history of fever, convulsion, trauma to head or neck and bowel bladder dysfunction. Cont …

8. No History of Diurnal variation , Seizure Trauma (Head, Neck, back) , Bowel & bladder involvement , Fever , Memory or cognitive loss , H eadache , Visual disturbance , Limb pain Cont …

9. No significant past illness . History of Past Illness

10. M arried All his family members are physically well No family history of same disease No family history of any inherited disease No consanguineous marriage of his parents Family History

11. Personal History Patient is Ex -smoker, non-alcoholic, non-betel nut chewer . No History of IV drug or substance misuse . Drug Histor y Patient is taking combination of linagliptin and metformin 2.5/500mg 12 hourly. Amlodipine 5mg in every night.

12. He is from higher middle class family . Immunization history Patient is not immunized under EPI schedule but immunized against COVID 19 (3 doses). Allergy History No history of allergy Socio economic History

13. GENERAL EXAMINATION

14. Appearance Ill looking, Anxious Body built A verage Co operation Co operative N utrition Below average Decubitus On choice Anaemia Absent Jaundice Absent Cyanosis Absent General examination

15. General Examination Clubbing Absent Oedema Absent Dehydration Absent Koilonychia Absent Leuconychia Absent Lymphnode Not Palpable Thyroid Not Palpable Bony Tenderness Absent

16. General examination Pulse 84 / min BP 130/80 mm/Hg Respiratory rate 18/min JVP Not raised Temperature 98.4 ℉ Weight 5 kg

17. SYSTEMIC EXAMINATION

18. NERVOUS SYSTEM

19. Higher psychic function: Behavior: Normal Emotional state: Normal Orientation of time, place, person: Normal Memory: Normal Intelligence: Average

20. Glossopharyngeal: Sluggish palatal Movement Pharyngeal reflex- Diminished Nasal Voice Vagus : Gag Reflex- Diminished Strength of Coughing – Diminished Hypoglossal: Generalized mild Tongue Wasting and f a sc iculation . Others: Normal. Cranial Nerve Examination

21. Upper & Lower Limb Examination INSPECTION UPPER & LOWER LIMB Muscle Atrophy- Thenar Hypothenar Forearm Mild atrophy present in the described parts. Dorsal Guttering Absent Fasciculation Absent Skin Change Absent Hair Change Absent Scar Mark Absent Pigmentation Absent Joint Deformity Absent

22. Upper Limbs: Bulk of Muscle: Reduced Right MUAC: 17cm Left MUAC: 18cm . Tone: De creased B/L . Power: MRC Grading Reflex: Supinator, Biceps, Triceps jerk- Diminished B/L . Sensory: All sensory modalities intact B/L LIMBS Upper Right Upper Left MRC Grade 3 /5 3 /5

23. Lower Limbs: Bulk of Muscle: Reduced Lower Right: 20cm Lower Left: 21cm . Tone: Increased B/L . Power: MRC Grading LIMBS Lower Right Lower Left MRC Grade 4 /5 4 /5 Patellar Clonus: Present B/L Reflex: Ankle, Knee Jerk- Exaggerated B/L Planter- Extensor B/L Sensory: All sensory modalities are intact B/L ,Including position and vibration. Gait: Steady

24. Cerebellar Function: Co ordination Finger nose test : Intact Heel shin Test : Intact Fundoscopy: Normal Spine Examination: Tenderness, Deformity, Reduced range of motions- Absent Meningeal signs: No signs of meningeal irritation .

25. Other System ic Examination Reveals no significant abnormality.

26. Mr. Prosanto , 7 0 years old married, diabetic, Hypertensive , ex smoker, non alcoholic hailing from Madhukhali , Faridpur got admitted to BSMMCH , Faridpur with the complaints of weakness in both upper limbs for last 1 years. Which is gradual in onset & progressive in nature. It was symmetrical. Initially it affected the distal muscles then proximal muscles were involved. The weakness is associated with muscle wasting in upper limb and not with any diurnal variations, easy fatiguability or any abnormal sensation. He also complains occasional twitching movement in different parts of the body. He has developed mild dysphagia to liquid and solid with nasal regurgitation for same duration. Salient Features

27. On general examination patient is anxious, body built is average. On nervous system examination, his intelligence is average, he has mild muscle wasting (Rt MUAC- 17cm, Lt MUAC-18cm, Rt LL- 20cm, Lt LL-21cm) with increased tone, decreased muscle power (U pper Limbs- 3 /5, L ower Limbs- 4 /5). All the deep reflexes of both lower limbs are exaggerated, planter extensor & clonus present bilaterally and upper limb is diminished. Salient Features

28. All his sensory modalities are intact. He has sluggish palatal movement, diminished pharyngeal reflex & nasal intonation, diminished gag Reflex. There is generali z ed tongue wasting & fa s ciculation but no deviation. His bowel & bladder habit is normal. Other system reveals no abnormality. Salient Features

29. PROVISIONAL DIAGNOSIS

30. Motor Neuron Disease

31. Differential Diagnosis

32. Cervical myeloradiculopathy

33. INVESTIGATIONS CBC: Hb%- 1 2 gm/dl WBC- 8 500/ cumm Platelet- 180,000/ cumm ESR- 20 mm in 1st hr RBS- 7 .4mmol/L S. Creatinine- 0.8mg/dl Urine R/E- Normal Thyroid Function Test- Normal VDRL ,TPHA: Negative Chest Xray P/A view : Normal

34. Chest X-Ray P/A View

35. MRI Cervical spondylosis C4-5 ; C5-6 ;C6-7 disco-osteophytic complex causing thecal indentation. At C4-5 there is left sided neural foramina narrowing. At C5-6 there bilateral significant neural foramina narrowing. C6-7 there is bilateral moderate neural foramina narrowing Screening whole spine: Screening of whole spine reveals mild diffuse degenerative changes. Lumber spondylosis with degenerative disease is noted.

36. MRI

37. MRI

38. MRI

39. EMG Chronic denervation and reinnervation changes in cervical and lumbosacral dermatomes , which favors anterior horn cell disorder

40. EMG

41. EMG

42. EMG

43. NCS Distal and proximal latencies was prolonged ,reduced amplitude and delayed conduction velocities from median ,ulnar, tibial, peroneal nerves Late response in F wave latencies from above nerve is prolonged IMPRESSION Electrophysiological study suggestive of motor neuropathy

44. NCS

45. NCS

46. Motor neuron disease Amyotrophic lateral sclerosis Final Diagnosis

47. Inj. Edaravon 1.5 mg/ml 1 amp IV in 200 ml normal saline 15 days apart for 3 cycles Tab. Rilutek 1 Tab orally 12 hourly after meal Tab. Ubiglow Q 1 Tab in each morning every day Treatment

48. Motor Neuron Disease Motor neuron disease (MND) is a neurodegenerative condition caused by upper and lower motor neurons in the spinal cord ,cranial nerve nuclei and motor cortex. Most cases are sporadic but 10% cases are familial and mutations in C9orf72 ,SOD1,VCP,FUS .Commonest form of MND is Amyotrophic lateral sclerosis. ALS characterized by a combination of upper and lower motor neurons . Average age of onset 65.

49. Limb muscle weakness, cramps , occasionally fasciculation . Disturbance of speech/ swallowing ( Dysarthria/ Dysphagia) Cognitive and behavioral abnormalities common ( similar to frontotemporal dementia.) SIGHNS Wasting and fasciculation of muscles. Pyramidal tract involvement ,causing spasticity, External ocular muscles and sphincters remains intact. No objective sensory deficit. Evidence of cognitive impairment with fronto temporal dominance Symptoms

50. Patterns of involvement in MND

51. Management Patient should be managed within a multidisciplinary service, including Physiotherapist Speech and occupational therapist Dietitians Ventilatory and feeding support . Neuroprotective agents : Riluzol , IGF 1, ASO , Vitamin E ,Co enzyme Q10 are used. Riluzol is a glutamate antagonist that may retard progression and prolong survival by about 2-3 months NIV significantly prolong survival and improves and maintains quality of life in people with ALS

52. Symptomatic Management Fatigue : Pyridostigmine , Amantadine Depression : SSRI , Venlafaxine , Amantadine Cramps: Quinidine, Vit E Fasciculation : Carbamazepine Spasticity : Baclofen , Tizanidine Joint pain: NSAID Insomnia : Zolpidem tartrate Respiratory failure : Bronchodilator

53. Even with treatment MND is relentlessly progressive . It is fatal within 3 to 5 years . Younger patient with early bulbar syndrome show a more rapid course. Prognosis is relatively better in Primary lateral sclerosis and progressive muscular atrophy. Cause of death usually respiratory failure and complications of immobility. Prognosis worse in Old age Female sex Bulbar onset Prognosis

54. New Light A new drug Edaravon has been approved by US FDA demonstrated a modest reduction in the trajectory of ALS worsening scale . Recently the combined oral administration of Phenylbutyrate and taururosodiol was reported to slow progression and prolong survival in ALS by improving function in Mitochondria and endoplasmic reticulum.

55. Thank Yo u