A CASE OF RECURRENT A CASE OF RECURRENT ALTERNATING ALTERNATING

TRANSIENT HEMIPARESISTRANSIENT HEMIPARESIS

Dr. Dr. ShunmugaShunmuga Arumugasamy.SArumugasamy.S

DNB ResidentDNB Resident

Railway Hospital, Perambur.Railway Hospital, Perambur.

�� 6 year old school going child.6 year old school going child.

�� Apparently normal till 3 yrs when she developed Apparently normal till 3 yrs when she developed

�� In EVENING:In EVENING:�� Rt. focal clonic seizures Rt. focal clonic seizures

---- 2 episodes2 episodes

---- In 1 hr interval In 1 hr interval

---- Each lasting 5 minEach lasting 5 min

---- Angle of mouth to Rt.Angle of mouth to Rt.

---- No loss of consciousnessNo loss of consciousness

---- No limb weaknessNo limb weakness

ØØ Advised to go to higher centre by a Pvt. practioner.Advised to go to higher centre by a Pvt. practioner.

�� Next day MORNING: Woke withNext day MORNING: Woke with

�� RtRt. sided limb weakness.. sided limb weakness.

�� Loss of speechLoss of speech

Admitted in Admitted in PvtPvt hospital in hospital in ChengalpetChengalpet

CT brainCT brain –– Infarct Infarct in in LtLt. . FrontoParietalFrontoParietalregion.region.

ECHO Heart ECHO Heart

Coagulation Profile NORMALCoagulation Profile NORMAL

Lipid ProfileLipid Profile

Baseline Inv (CBC,LFT,RFT) Baseline Inv (CBC,LFT,RFT) -- NORMALNORMAL

In a week

nn Speech improved in 6 days with slight Speech improved in 6 days with slight stuttering.stuttering.

nn Minimal distal weakness continued.Minimal distal weakness continued.

In ICH, In ICH, EgmoreEgmore::

MRI BRAINMRI BRAIN -- Similar findings.Similar findings.

Aspirin, Aspirin, CarbamazepineCarbamazepine –– advised.advised.

New symptomsNew symptoms

�� 2 ½ years later2 ½ years later

�� 5 months ago…5 months ago…

�� LeftLeft sided weakness sided weakness –– sudden onset; in MORNING sudden onset; in MORNING �� Not able to walk.Not able to walk.

�� Unable to hold objects in Lt hand.Unable to hold objects in Lt hand.

�� Lasted for 5 min Lasted for 5 min àà then improved.then improved.

�� No loss of consciousness.No loss of consciousness.

�� Similar episodic weakness Similar episodic weakness –– in EVENINGin EVENING�� Same sideSame side

�� Now speech also affected.Now speech also affected.

�� Lasted 10 min Lasted 10 min ----> then improved.> then improved.

�� Admitted in our Hospital.Admitted in our Hospital.

�� O/EO/E

§§ ConsciousConscious

§§ Oriented to time, place & personOriented to time, place & person

§§ ComfortableComfortable

�� Vitals: Normal.Vitals: Normal.

�� No Neurocutaneous markers seen.No Neurocutaneous markers seen.

CNS ExaminationCNS Examination

�� Higher Mental Functions:Higher Mental Functions:�� Speech Speech –– AphasicAphasic

�� Cranial Nerves:Cranial Nerves:�� 77thth(Lt(Lt)) –– UMN type Facial palsyUMN type Facial palsy

�� Motor System:Motor System:

§§ BulkBulk

§§ Tone NormalTone Normal

ØØ PowerPower

§§ UL UL -- 3/5 (L)3/5 (L)

-- 4/5 (R)4/5 (R)

§§ LL LL -- 5/5 ( R & L)5/5 ( R & L)

�� Reflexes:Reflexes:

§§ Superficial : Present; Plantar Superficial : Present; Plantar –– Left Left

§§ Right Right --

§§ DTR : Exaggerated (L) ; Normal (R)DTR : Exaggerated (L) ; Normal (R)

�� No involuntary movements.No involuntary movements.

�� Gait Gait –– Hemiplegic gait.Hemiplegic gait.

�� Spine and Cranium Spine and Cranium –– Normal.Normal.

�� Bladder and Bowel Bladder and Bowel –– Normal. Normal.

�� CVS, RS, Abdomen CVS, RS, Abdomen -- Normal.Normal.

�� Provisional Diagnosis :Provisional Diagnosis :

Rec. Alternating Transient Rec. Alternating Transient HemiparesisHemiparesis

DDDD

1. 1. ProcoagulantProcoagulant states.states.

2. Carotid 2. Carotid thromboembolicthromboembolic diseasedisease3. Blood 3. Blood dyscrasiasdyscrasias and strokeand stroke4. 4. VasculitidesVasculitides

5. Basilar artery thrombosis5. Basilar artery thrombosis

6. Metabolic disorders (MELAS, 6. Metabolic disorders (MELAS, HomocysteinuriaHomocysteinuria, Methyl , Methyl malonicmalonic acidemiaacidemia, , FabryFabry disease)disease)

7. Cerebral Aneurysms7. Cerebral Aneurysms

INVESTIGATIONSINVESTIGATIONS

ØØ Pr C, Pr S, ATPr C, Pr S, AT--III, Factor V Leiden III, Factor V Leiden –– Normal.Normal.

ØØAntiPhospolipidAntiPhospolipid, , AnticardiolipinAnticardiolipin, Lupus , Lupus anticoagulant antibodies anticoagulant antibodies –– Negative.Negative.

ØØBlood Lactate Blood Lactate –– Normal.Normal.

ØØCarotid DopplerCarotid Doppler –– Normal.Normal.

ØØPHPH –– NormalNormal

ØØESRESR –– NormalNormal

ØØSerum homocysteine levels Serum homocysteine levels -- NormalNormal

CT angiography CT angiography -- Revealed the Revealed the DiagnosisDiagnosis

n The petrous, cavernous and supraclinoid parts of right ICA appears thin calibered.

n Left ICA reveals critical stenosis beyond its P1 segment. Distally reformatted by collaterals.

n Anterior cerebral arteries appear normal on both sides.

n Basilar artery and both vertebral arteries are normal.

n ‘Puff of Smoke’ appearance of Collaterals.

DiagnosisDiagnosis

MOYA-MOYA DISEASE

Neurosurgical intervention: (VHS,Adyar)

§ Revascularization procedure

((GaleoGaleo arachnoidarachnoid synanastamosissynanastamosis) ) with with multiple burr holesmultiple burr holes done.done.

ØØ CarbamazepineCarbamazepine and Aspirin continued.and Aspirin continued.

Ø Physiotherapy and Speech therapy continued.

Presently…Presently…

Able to walk.Able to walk.

Able to eat with Rt. hand with minimal Able to eat with Rt. hand with minimal spilling.spilling.

Left side weakness improved.Left side weakness improved.

Continues to be Aphasic.Continues to be Aphasic.

MoyaMoya--Moya DiseaseMoya Disease

Moya moya diseaseMoya moya disease was first described in Japanwas first described in Japan in 1957.

Progressive steno-occlusive disease at terminal portions of the bilateral internal carotid arteries with the development of "moyamoya vessels" as collateral channels of circulation.

Moya moya syndrome - same angiographic appearance associated with conditions such as arteriosclerosis, autoimmune disease, Down syndrome, head trauma, meningitis, neurofibromatosis type 1, and previous radiation therapy.

Bilateral concentric stenosis or occlusion of the Bilateral concentric stenosis or occlusion of the distal distal internal carotid arteriesinternal carotid arteries and the and the proximal anterior proximal anterior and middle cerebraland middle cerebral arteriesarteries are consistent are consistent pathologic lesions.pathologic lesions.

Rarely, the posterior cerebral artery or other vessels Rarely, the posterior cerebral artery or other vessels of posterior circulation are involved.of posterior circulation are involved.

Diffuse collateral reticulate network of vessels (Diffuse collateral reticulate network of vessels (moya moya moya vesselsmoya vessels) ) -- HallmarkHallmark of the disease. of the disease.

Etiology:Etiology:

§§ Unknown Unknown -- though myriad though myriad bacterial, bacterial, environmental, genetic, and viralenvironmental, genetic, and viral causes causes have been theorized.have been theorized.

§§ HLA class II association +HLA class II association +

§§ FamilialFamilial

Extracranial involvement: Extracranial involvement:

Renal, pulmonary, and coronary vessels.Renal, pulmonary, and coronary vessels.

DiagnosisDiagnosis

Cerebral angiographyCerebral angiography -- Gold standard.Gold standard.

MRI and MRA MRI and MRA –– other methods of diagnosis other methods of diagnosis but inferior to Cerebral Angiogram.but inferior to Cerebral Angiogram.

Cerebrospinal fluid exmn. Cerebrospinal fluid exmn. –– UnremarkableUnremarkable

EEG EEG -- can be abnormal but are usually can be abnormal but are usually nonspecificnonspecific..

Management Management

No known treatment is curative.No known treatment is curative.

Medical:Medical:

§§ Anticoagulant and antiplatelet agents have shown Anticoagulant and antiplatelet agents have shown no remarkable benefit.no remarkable benefit.

§§ Corticosteroids Corticosteroids –– not beneficial.not beneficial.

Surgical treatment:Surgical treatment:

Main modality of Treatment.

§§ To manage the hemorrhagic and ischemic consequences of To manage the hemorrhagic and ischemic consequences of moyamoyamoyamoya disease.disease.

§§ Revascularization procedures.Revascularization procedures. DirectDirect

Indirect Indirect

DirectDirect –– usually in adults;usually in adults;

§§ Superficial temporal artery to middle cerebral Superficial temporal artery to middle cerebral arteryartery bypassbypass or or

§§ Middle meningeal artery to middle cerebral artery Middle meningeal artery to middle cerebral artery bypass.bypass.

IndirectIndirect -- traditionally used in children.traditionally used in children.

Galeo Arachnoid synanastamosis by multiple burr holes. Encephaloduroarteriosynangiosis (EDAS)

Encephalomyosynangiosis (EMS)

Omental Transposition

Encephalomyoarteriosynangiosis (EMAS)

Increase perfusion in the Increase perfusion in the cerebrovascularcerebrovascular territory of the territory of the middle cerebral arterymiddle cerebral artery..

Do not substantially effect the circulation in the fields of the Do not substantially effect the circulation in the fields of the anterior or posterior cerebral arteriesanterior or posterior cerebral arteries . .

Literature reviewLiterature review

nn Nigel Peter Nigel Peter SymssSymss, , RavindranathRavindranath kapukapu et al. et al. Multiple Multiple burr hole surgery as a treatment modality for pediatric burr hole surgery as a treatment modality for pediatric moyamoyamoyamoya disease. disease. J J PediatrPediatr NeurosciNeurosci. Jul . Jul 2010;5 2010;5 (2):115(2):115--120120..

nn 22--yryr--old boy presented with recurrent episodes of old boy presented with recurrent episodes of TIAs with seizures since the age of 6 months TIAs with seizures since the age of 6 months –– MoyaMoyaMoyaMoya. EDAS done. Now no TIA like episodes. . EDAS done. Now no TIA like episodes. Y.C. Y.C. ManjunathaManjunatha and and ArunArun Kumar Gupta. Kumar Gupta. IJP IJP --JUL.2010;77(7):817.JUL.2010;77(7):817.

Thank YouThank You