Case presentation

1. Case presentation 醫學七 蕭皓天 8831121 Supervisor 許明欽醫師

2. Basic data • Name：羅X化 • Age：43 y/o • Gender：male • Dominant：Right hand • Admission date：94/10/20

3. Chief complaints • Severe headache for 1 months with progression

4. Present illness • Well before • One month ago：occipital headache suddenly after sitting up for 10 mins.

5. Present illness • Accentuated：upright position • Relieved：recumbent • Timing：seconds or minutes after sitting or standing • Severity：made him didn’t like to get up from bed

6. Present illness • Frequency：every times when upright position • Quality：throbbing • No aura, no photophobia, no phonophobia

7. Present illness • 9/19：Our ER, brain CT：normal • Recent one week： more severe, extended to vertex • Nausea, vomiting and hiccup • 10/20：Dr.許’s OPD • Admission

8. Social History • Smoking：1 ppd/day • Alcohol (-) • Betal nut (-)

9. Other History • Hypertension (-) • Diabetes mellitus (-) • Operative history (-) • Head traumatic history (-) • Lumbar puncture history (-) • Allergy：nil • Family history：not contributory

10. Physical examination • Moderate nutritional and well-developed, looks inpatient, irritant and general weakness • T/P/R：36.3C, 80/min, 18/min, BP：134/77 mmHg • HEENT：normal • Neck：no bruit, no jugular vein engorgement, no thyroid enlargement

11. Physical examination • Chest：no chest wall deformity, clear BS • Heart：regular heart beat, no murmur • Abdomen：soft, flat, no tender, normoactive bowel sound • Extremities: no cyanosis, no pitting edema

12. Neurological examination • Consciousness：alert, oriented • No overt high cortical dysfunction • Cranial nerve：intact • Eye：no papilledema • Neck：supple, no Kernig sign, no Brudzinski sign

13. Neurological examination • Pyramidal system: • No atrophy • Muscle power：Full MRC grading bilaterally • Symmetric normoreflexia • No pathologic long tract sign • Extrapyramidal system: • No rigidity, no spasticity, no tremor • Sensory system：intact

14. Neurological examination • Cerebellum： • Finger to nose: Smooth • Romberg test: Stable, tandem gait：ok • Autonomic system： • palpitation(-), nausea(+), vomiting(+), constipation(-), diarrhea(-) • No sphincter dysfunction

15. Lab data • GPT/ALT 108 IU/L • BUN 24 mg/dl • TCH 227 mg/dl • LDL-C 167 mg/dl • TG 237 mg/dl

16. Headache What kind of headache suggests a serious underlying disorder ?

17. Headache Symptoms That Suggest a Serious Underlying Disorder • "Worst" headache ever • First severe headache • Subacute worsening over days or weeks • Abnormal neurologic examination • Fever or unexplained systemic signs • Vomiting precedes headache • Induced by bending, lifting, cough • Disturbs sleep or presents immediately upon awakening • Known systemic illness • Onset after age 55 Headache presents as above indicates intra-cranial tumor, hemorrhage (Ex.SAH) meningitis…etc

18. Tentative diagnosis • Acute orthostatic headache

19. Isolated orthostatic headache DDx • Intracranial hypotension CSF volume depletion - spontaneous  most likely！ - dural puncture - surgery - penetrating trauma • Colloid cyst of 3rd ventricle  Brain CT(-)

20. After admission… MRI was arranged immediately

21. 10/20 Brain MRI T1WI (isointensity)

22. 10/20 Brain MRI T2WI (hyperintensity)

23. Brain CT 9/19 vs 10/21

24. Clinical course • Subdural hemorrhage diagnosed by neuroimage => Neurosurgeon suggested operation • He and his family refused • Discharged against advice on 94/10/21

25. Question 1 According to the clinical picture, spontaneous intracranial hypotension leading to bil. SDH was suspected, were there similar cases in the world？

26. Intracranial hypotension • A generalized sagging of the brain with downward displacement of the cerebellar tonsils • Downward displacement leads to bringing vein break  SDH

27. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clinic Proceedings, 72:400-413, 1997. • Patients had diffuse meningeal enhancement, 69% had evidence of subdural CSF collections and 62% showed a descent of the brain

28. 31 y/o male, previously healthy • Headache：occipito-parieto-temporal • Accentuated：upright position • Relieved：recumbent • Getting worse and worse, nausea, vomiting • NE, lab：normal

29. Subdural effusion Diffuse dural thickening with enhencement

30. CSF：low pressure (10cm H2O) • SIH was diagnosed • Consciousness↓, epidural blood patch and surgical drainage was performed • Consciousness recovered and headache subsided

31. .Spontaneous intracranial hypotension associated with bilateral chronic subdural hematomas – Case reportNeurol Med Chir (Tokyo). 2000 Sep;40(9):484-8. • 34-year-old female：severe postural headache and meningism. • MRI：diffuse pachymeningeal enhancement. • Bil. chronic SDH 4 weeks after the onset of the symptoms. MRI showed descent of the midline structures of the brain.

32. Spontaneous intracranial hypotension associated with bilateral chronic subdural hematomas – Case reportNeurol Med Chir (Tokyo). 2000 Sep;40(9):484-8. • An uncommon and probably unrecognized condition, because of the usually benign course. • However, SIH is not entirely benign.

33. Spontaneous intracranial hypotension associated with bilateral chronic subdural hematomas – Case reportNeurol Med Chir (Tokyo). 2000 Sep;40(9):484-8. • SIH should be considered  no identifiable risk for ICH, particularly in young patients. • Neurosurgical intervention may be required for the underlying cerebrospinal fluid leak and subdural effusion

34. Question 2 Why MRI was performed first？

35. Intracranial hypotension • Diffuse meningial thickening - Compensatory venous engorgement secondary to the chronically low CSF volume ▲ Subdural effusions

36. Level II • CT versus MRI • CT：very limited value. Usually normal and only quite infrequently may show subdural fluid collections or increased tentorial enhancement Sensitivity 85%, specificity 65% • MRI：diffuse pachymeningeal enhancement, which is the most common MRI abnormality. Sensitivity > 95%, specificity > 90%

37. Return to our patient • MRI was important for diagnosis of intra-cranial hypotension • There were several cases like this patient in the world • Spontaneous intra-cranial hypotension’s prognosis is good. It’s a pity that he refused further treatment such as epidural patch and operation

38. Question 3 Should our ER perform CT？

40. In patients with atypical headache patterns, a history of seizures, or focal neurologic signs or symptoms, CT or MRI may be indicated  Class III

41. Historical or physical abnormalities are not sensitive for intracranial process, but abnormal physical or historical findings increase the likelihood of positive CT findings  Class II

42. Study supports the importance of a neurologic examination; however, 29 of 34 Patients with focal findings didn’t have positive CT findings, and 4% of patients with normal neurologic examination findings had positive CT results Class II

43. Clinical findings and historical findings had a low positive predictive value but absence had a high negative predictive value  Class II

44. Conclusion • Atypical headache with either abnormal physical, neurologic, historical findings or a history of seizures  CT or MRI was indicated • CT was not very essential for this patient

45. Comment • 李宜恭主任：SIH診斷的golden standard？ Ans：Lumbar puncture. 許明欽醫師：這個病人為什麼沒有做，是因為本來打算在手術完之後，做lumbar puncture確立診斷，同時加做cisternography來判斷到底是哪裡在漏CSF？(不先做lumbar puncture是擔心有herniation的危險性)只是病人要求出院，這些study工作才未完成。

46. Comment • 李宜恭主任：對這個病人來說，做CT還是有必要，因為他還是符合危險頭痛其中幾項，在沒辦法完全排除出血或腫瘤的前提下，做CT是可接受的。而且這個病人症狀有緩解(休息和bed rest本來就會讓病人覺得恢復)，沒有繼續留下來觀察尚屬合理。

47. Comment • 許明欽醫師：大多數SIH會自己好，因此ER讓病人出院是合理處置。重點還是在於病人症狀有減輕，影像學又沒有finding。

48. Comment • Int. 傅斯誠：bil. SDH在什麼情形下會發生？ 許銘欽醫師：通常還是以trauma最常見。

49. Thanks！