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A 3-year-old girl with frequent right focal seizure while irritability or excitement for 2 months 高雄長庚醫院小兒神經科 Case discussion 1.

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Presentation on theme: "A 3-year-old girl with frequent right focal seizure while irritability or excitement for 2 months 高雄長庚醫院小兒神經科 Case discussion 1."— Presentation transcript:

1 A 3-year-old girl with frequent right focal seizure while irritability or excitement for 2 months 高雄長庚醫院小兒神經科 Case discussion 1

2 General data Sex: female Birth date: Admission date: Discharge date: Caregiver and information source: mother

3 Chief complaint: Right focal seizure for 20 minutes on the day of admission

4 Present Illness This 3-year-2-month-old girl visited OPD on due to frequent right focal seizure while irritability or excitement in recent 2 months, almost 10 episodes a day Drooling, eye staring, conscious disturbance, right extremities clonic movement after crying. Post-event right hemiparesis and dysarthria was associated.

5 She also got URI symptoms for 1 week. Her body temperature was up to 41 °C for 1 day. Then frequent right focal seizure followed. Her seizure pattern: –Right hand clonic → right arm clonic → loss of consciousness → right hemifocal twitching → postictal right extremities weakness

6 The postictal limb paralysis persisted long, for around half a day. There was no vomiting, nor diarrhea. Further management will be arranged.

7 Past history –Denied systemic disease –Denied past surgical history Birth history –G2P2, GA: 38 wks, BBW: 2350gm (small for gestational age), via C/S due to pre-C/S, no DOIC, no perinatal insult, slow in growth after delivery Newborn screen: – normal Vaccination: as scheduled Family history –Denied any inherited disease

8 Physical Examination T:38/ ℃ P:128/min R:28/min BP:93/48/mmHg ( 下肢 ) 身高 :90CM (3rd percentile) 體重 :12KG (10th percentile) HEENT – Head: no trauma, no deformity – Conjunctiva: not pale – Sclerae: not icteric – Throat: injected – Tonsil: not enlarged Neck: supple, no nuchal rigidity Chest – symmetrical expansion, no retraction – no rale, no wheezing Heart –Regular heart beat without murmur Abdomen: –No hepatosplenomegaly Extremities: no edema, capillary refilling< 2 seconds Other finding: no skin stigmata

9 Neurological Examinations Conscious level: E4V5M6 Cranial nerve evaluation –CN I: not checked because of incooperation –CNII: not impaired in visual acuity; light reflex intact –CNIII.IV, VI: not limited in eye movement –CNVII: absence of right nasolabial fold, presence of bilateral forehead wrinkle, closure of right eyelid (+), → right central facial palsy

10 –CNVIII: auditory acuity is not checked –CN IX&X: hypernasality, difficulty in swallowing, easily chocking –CNXI: shoulder shrug OK, SCM muscle power OK –CNXII: no tongue deviation Muscle power assessment: RL: 3 points RA: 3 pointsLA: 5 points LL: 5 points

11 Deep Tendon Reflex: Barbinski sign: dorsiflexion, right foot Right elbow: brisk Right knee, ankle: brisk Left elbow: normal Left knee, ankle: normal

12 Lab Data on Admission Hematogram –WBC 10200/CMM –Hb 12.4 g/dL –MCV 81.1 FL –PLATELET /CMM –SEGMENT 84.5 % –LYMPHOCYTE 8.4 % –MONOCYTE 6.7 % –EOSINOPHIL 0.3 % –BASOPHIL 0.1 % Biochemistry –GLU 111 mg/dL –AST 35 U/L –ALT 15 U/L –CA(B) 8.8 mg/dL –NA(B) 140 meq/L –K(B) 3.9 meq/L –CRP 23.1 mg/L Interpretation The hematogram and biochemistry revealed within normal range

13 Lab Data on Admission (II) ESR 20 MM/HR (0-15) C mg/dL (90-180) C mg/dL (10-40) ANA ( 1:40 ) B2-microglobulin ug/L ( ) MYCO-IgG NEGATIVE MYCO-IgM NEGATIVE EB-VCAG NEGATIVE EB-VCAM NEGATIVE HSV-1 IgG NEGATIVE HSV-2 IgG NEGATIVE VIRUS(TH) NO VIRUS ISOLATED U/A: negative

14 Tentative diagnosis Acute ischemic stroke Upper respiratory tract infection Focal seizure and neurologic deficits (transient ischemic attack) usually developed after hyperventilation, moyamoya disease should be considered

15 Brain CT (on 5/25): R/O meningitis with left frontal brain edema.

16 Sleep EEG: This is an abnormal EEG during sleep that demonstrated left frontocentral cortical dysfunction.

17 Brain MRI + MRA Demostration

18 Axial T1

19 Axial T2

20 DWI

21 ADC

22 MRA

23 MRI of Brain report: Conclusion: 1. Lt F-T-P recent infarct lesion 2. Non-opacification of Lt MCA 3. Moyamoya disease is highly suspected

24 Hospitalization Course (Day 1)Admission to ward, basic blood sampling (Day 2) Right focal (arm) seizure in the midnight, duration 10 minutes, 3 episodes, no loss of consciousness → Valium iv, check BP q8h → Emergent brain CT with enhancement → Ampicillin + cefotaxime ivf → transfer to PICU at 21:00, due to low BP (right leg: 67/33 mmHg, right arm: 119/57 mmHg) and drowsy consciousness (Day 3) Patient still cannot walk, right side weakness → Brain MRI + MRA, Cardiac 2D echo for differential hypertension (R’t arm: 143/74, R’t leg: 87/54 mmHg) → EEG, check mycoplasma, EBV, HSV IgG/IgM

25 (Day 4)Patient still had fever, up to 38.5 °C, no seizure so far Consult Neurosurgeon (Day 5) Patient fever subsided, no seizure so far Consult CV specialist, arrange abdominal CT + angiography → Coarctation of descending aorta Transfer to ward (Day 8) Consult rehabilitation specialist Aspirin (100mg) ¼# po QD, then DC on (Day 18) Discharge to 6-20 Re-admission for balloon angioplasty for coarctation of descending aorta 97-8Family visited VGH Taipei for surgical intervention

26 Balloon angioplasty

27 Discussion about Moyamoya disease

28 An uncommon cerebrovascular disease In Japan annual prevalence and incidence: 3.16 and 0.35 per 100,000 female to male ratio: 1.8 characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches (within the circle of Willis) Lancet Neurology 2008, Moyamoya Disease

29 Associated with the development of dilated, fragile collateral vessels at the base of the brain, termed moyamoya vessels.

30 The incidence is high in east Asia, and familial forms account for about 15% of patients with this disease. two peaks of age distribution –at 5 years –at about 40 years. Definite cases of moyamoya disease are diagnosed in patients with bilateral lesions, whereas patients with unilateral lesions are diagnosed as probable cases.

31 Clinical Presentation in Child with Moyamoya Disease Most pediatric patients have ischemic attacks; adult patients can have ischemic attacks, intracranial bleeding, or both. The cerebral ischemia usually in the territory of the ICA, particularly in the frontal lobe. Most patients present with focal neurological signs, such as dysarthria, aphasia, or hemiparesis.

32 Other atypical symptoms such as syncope, paraparesis, visual symptoms, or involuntary movements (eg. Chorea). Some pediatric patients develop intellectual impairment owing to frontal lobe ischemia, infarct, or both. Ischemic attacks are induced by hyperventilation, crying or playing a wind instrument for example

33 Etiology unknown Differential diagnosis –atherosclerosis –autoimmune disease –Meningitis –Brain neoplasm –Down’s syndrome –Neurofibromatosis –Head trauma –Irradiation to the head so-called moyamoya syndromes

34 Genetic factor is important, rather than environment. –polygenic or autosomal dominant mode with a low penetrance –Microsatellite linkage analysis : chromosomes 3, 6, 8, and 17 Infectious role –infection in the head and neck might be implicated in the development of moyamoya disease

35 Histopathological findings fibrocellular thickening of the intima, irregular undulation (waving) of the internal elastic lamina, and attenuation of the media.

36 Growth factors and adhesion molecule increase in patient’s CSF –basic fibroblast growth factor –soluble vascular-cell adhesion molecule type 1 –intercellular adhesion molecule type 1 –E-selectin –hepatocyte growth factor Cytokine increase in the CSF indicated inflammatory process in CNS The lancet of neurology, 2008

37 Angiogenic proteins –formation of collateral circulation after surgical revascularization with indirect bypass.

38 Treatment Extracranial–intracranial arterial bypass, –anastomosis of the superficial temporal artery to the middle cerebral artery –Frontal burr hole

39 The Association of Moyamoya Disease and Congenital Heart Disease Result: Five patients with moyamoya syndrome and structural congenital heart disease –CoA in 3 patients, in association with a ventricular septal defect (1 patient), aortic and mitral valve stenoses (1 patient), and tetralogy of Fallot (1 patient). –The other two had Tetralogy of Fallot and a large paramembranous ventricular septal defect pediatrics 1998

40 In these 5 patients, moyamoya syndrome was diagnosed after surgical intervention for congenital heart disease— 6 mo in 1, 2yr in 3, and 6 yr in 1 Stroke in 3; seizure in 2

41 Conclusion Moyamoya syndrome should be considered in the differential diagnosis of seizures and stroke in patients with structural congenital heart disease Based on our experience and literature review, blood pressure of 4 limbs should be measured routinely in moyamoya disease

42 The end


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