A case of a 14 year old boy with limb weakness.pptx

1. Dhaka Medical College Hospital Weekly Grand Round Presentation A CASE OF A 14 YEAR OLD BOY WITH LIMB WEAKNESS MEDICINE UNIT VIII Department of Medicine

2. ABOUT THE PATIENT 01

3. PARTICULARS OF THE PATIENT AGE GENDER LOCATION 14 Male Nakla, Sherpur MD MUNNA OCCUPATION Madrasa Student

4. PRESENTING COMPLAINTS 02

5. PRESENTING COMPLAINTS Unable to swallow and speak for 5 days Confusion and excessive sleepiness for 15 days Weakness of all four limbs for 20 days 01 02 03

6. THE HISTORY 03

7. HISTORY OF PRESENT ILLNESS ● According to the statement of his sister, he was reasonably well one month back. ● His illness started with weakness in the right lower limb, initially associated with pain. ● The pain subsided and the weakness progressively involved the right side of the trunk, right upper limb, the left lower limb and then to left upper limb over a period of 3 weeks. He did not complain any numbness.

8. HISTORY OF PRESENT ILLNESS ● During this period, he was remarkably sleepy, tired, had difficulty in walking due to loss of balance. He frequently complained of headache. ● With these complaints he repeatedly consulted Paediatric Neurology Department in BSMMU on OPD basis first and then got admitted. ● Medical records show that he had ataxia, nystagmus, hypertonia, hyperreflexia and extensor plantar reflex with urinary incontinence at the time of admission on September, 2022.

9. ● During the 12 days of admission his condition deteriorated progressively. He developed dysphagia, dysarthria, dysphonia and GCS level went down from 15 to 9. So, he was referred to DMCH. ● While in DMCH his conditions initially worsened, GCS dipped down to 3. Then his condition started to improve and GCS was 13 by day 5 of admission. His urinary incontinence and dysphagia resolved. Significant muscle weakness persisted. HISTORY OF PRESENT ILLNESS

10. ● Now the patient can walk albeit with support, GCS persists at 15. ● He had no fever or seizure at any point. There is no history of any preceding illness or contact with TB patient. His bowel habit was normal throughout. HISTORY OF PRESENT ILLNESS

11. OTHER RELEVANT HISTORY HISTORY OF PAST ILLNESS Nothing of significance PERSONAL HISTORY Active teenage student. Plays football. Non-smoker, no history of narcotic use SOCIOECONOMIC HISTORY Comes from a lower middle class family FAMILY HISTORY Patient has an elder sister. Both parents alive and well (No consanguinity). No history of similar illness in either side of the family. TRAVEL HISTORY No travel history outside of Dhaka and Sherpur in last one year IMMUNISATION HISTORY

12. PHYSICAL EXAMINATION 04

13. An ill-looking young man who is very drowsy and confused 04

14. PHYSICAL EXAMINATION: GENERAL PERFORMED ON 29 SEP Appearance Semi-conscious, ill-looking, vacant expression Anemia/Jaundice/Cyanosis Absent Clubbing Absent Koilonychia/ Leukonychia Absent Edema Absent Lymphadenopathy Absent BP 100/70mmHg Pulse 72/min Temperature Normal Respiration Normal, SpO2 100% on air

15. SYSTEMIC EXAMINATION Detailed neurological examination was difficult due to the patient’s fluctuating level of consciousness. The most detailed neuro exam could be carried out on 03 OCT Other systemic examination yielded no positive findings

16. Patient kept drifting off while examination

17. CRANIAL NERVES Could not be performed due to low level of consciousness MOTOR RU RL LU LL Tone Strength Could not be assessed due to low level of consciousness DEEP TENDON REFLEXES Biceps Triceps Supinator Knee Ankle R All are exaggerated without clonus L SYSTEMIC EXAMINATION: NERVOUS SYSTEM PERFORMED ON 29 SEP GCS 7/15 (E4V2M1) PLANTAR REFLEX Flexor Bilaterally

18. CRANIAL NERVES II, III, IV, VI Significant palsy in right lateral gaze, right eye; upward gaze, both eyes Nystagmus on both lateral gazes. Saccadic eye movement with broken pursuit V Sensations Intact, Significant weakness, symmetrical VII Symmetrical weakness IX, X Could not cough or open mouth wide enough XI, XII Symmetrical Weakness MOTOR RU RL LU LL Tone Strength 1/5 2/5 1/5 2/5 DEEP TENDON REFLEXES Biceps Triceps Supinator Knee Ankle R All are exaggerated without clonus L SYSTEMIC EXAMINATION: NERVOUS SYSTEM PERFORMED ON 3 OCT GCS 14/15 (E4V4M6)

19. PLANTAR REFLEX Flexor Bilaterally COORDINATION Could not perform due to weakness GAIT Could not perform due to weakness SENSORY SYSTEM All sensory modalities intact throughout the body SYSTEMIC EXAMINATION: NERVOUS SYSTEM PERFORMED ON 3 OCT HIGHER PSYCHIC FUNCTION MMSE Score 20/30 OPHTHALMOSCOPY Normal findings

20. TIMELINE OF PROGRESSION 05

21. TIMELINE OF DISEASE PROGRESSION First symptom appears: Right lower leg pain 27 AUG Developed headaches and sleepiness 30 AUG BSMMU Outpatient consultation 2 SEP Leg pain and headache settles. Sleepiness remains. Notices weakness in right leg 4 SEP Develops urinary incontinence. Weakness worsens 15 SEP

22. Repeat BSMMU outpatient consultation. Exaggerated DTR, Babinsky sign, Nystagmus, Ataxia found on exam. Admission. 17 SEP Dysphagia appears. GCS dips to 9. ICU advice anticipating respiratory muscle paralysis 25 SEP Admitted to DMCH. GCS 7. Plantar returns to flexor. 29 SEP Condition worsens. GCS goes down to 3. No respiratory paralysis. 30 SEP Level of consciousness improves but fluctuating. GCS 7 (E4V2M1) 01 OCT TIMELINE OF DISEASE PROGRESSION

23. Condition keeps improving. Muscle power MRC 1 /2 across limbs. GCS 12/15. Keeps drifting off. 02 OCT NG removed, can swallow liquids. Incontinence resolves. Party decides to leave 03 OCT Consciousness level worsened, not arousable for most of the day. Contacts DMCH and advised to admit. Improves spontaneously late in the evening, stays home. 06 OCT Can walk with support. Drowsiness decreased but still significant. 09 OCT Comes in for follow up? 12 OCT TIMELINE OF DISEASE PROGRESSION

24. SALIENT FEATURES A 14 year old fit madrasa student develops leg pain and weakness Pain subsides but the weakness ascends and affects all limbs At the same time he develops headache and excessive sleepiness He eventually developed ophthalmoplegia, ataxia, dysphagia, Dysarthria At no point was there any fever. There was no history of preceding illness Over the course of a month his weakness and drowsiness worsened Examination revealed hypertonicity and hyperreflexia. Plantar initially extensor, then flexor Sensory modalities were intact throughout

25. Provisional Diagnosis Autoimmune Encephalitis

26. DIFFERENTIAL DIAGNOSIS BICKERSTAFF BRAINSTEM ENCEPHALITIS A NEUROMYELITIS OPTICA C B D GUILLEN BARRE SYNDROME TB ENCEPHALITIS

27. INVESTIGATIONS 07

28. INVESTIGATIONS HEMATOLOGY (28 Aug 2022) Haemoglobin 13.6 g/dl ESR 05 mm in 1st hour Total Count 8200/ cm3 Differential Count Neutrophil: 55% Lymphocytes: 34% Monocytes: 07% Eosinophil: 04% Circulating Eosinophils 328/cm3 Platelet Count 2,82,000/cm3 RBC Count 5.02 m/microlitre Hematocrit 42.3% HEMATOLOGY (20 Sep 2022) Haemoglobin 13.3 g/dl ESR 04 mm in 1st hour Total Count 11000/ cm3 Differential Count Neutrophil: 92% Lymphocytes: 06% Monocytes: 02% Eosinophil: 00% Circulating Eosinophils 328/cm3 Platelet Count 3,82,000/cm3 RBC Count 4.07 m/ml Hematocrit 41.2%

29. INVESTIGATIONS BIOCHEMISTRY CRP 0.22 mg/L Creatinine 0.83 mg/dL AST 10 U/L S Electrolytes Na+ 138.00 mmol/L K+ 4.62 mmol/L Cl- 102.00 mmol/L SEROLOGY ASO Titre 200 IU/mL AntiDsDNA 11.0 U/mL ANA Negative SARS-COV2 Negative HBsAg Negative

30. INVESTIGATIONS CSF STUDY Volume 1 ml Color Watery Appearance Clear WBC Count 03 Cells/ cm3 Neutrophils 20% Lymphocyte 80% CSF STUDY Glucose 6.1 mmol/L Protein 1.0 gm/L Chloride 115mmol/L Gram Stain No organism seen AFB No organism seen ADA 15.0 u/L GeneXpart Negative

31. INVESTIGATIONS IMAGING MRI BRAIN No abnormalities seen MRI Spine No abnormalities seen AUTOIMMUNE ENCEPHALITIS PANEL NMDA Negative AMPA Negative GABA-B Negative LGi-1 Negative Anti GQ1b Ab Pending NEUROPHYSIOLOGICAL STUDIES EEG Normal NCS and EMG No peripheral demyelination or axonal damage. During voluntary activation there is no interference or activation suggestive of central cause of motor weakness

32. The Diagnosis Bickerstaff’s Brainstem Encephalitis

A case of a 14 year old boy with limb weakness.pptx

A case of a 14 year old boy with limb weakness.pptx

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