Stroke Syndromes And Imaging Syed F. Ali.

Syndrome: Ataxic Hemiparesis Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: R sided Weakness – upper and lower extremity R sided Ataxia – arm and leg Extensor plantar response No facial involvement or dysarthria Syndrome: Ataxic Hemiparesis Anatomy: Cerebral Hemisphere: Posterior limb of internal capsule Pons: Basis pontis Vascular: Middle cerebral artery: Small penetrating arteries Basilar artery: Small penetrating arteries

Syndrome: Gertsmann Syndrome Case: 78 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Agraphia, Acalculia, Right-left confusion, Finger agnosia (inability to recognize fingers), Ideomotor apraxia Syndrome: Gertsmann Syndrome Anatomy: Cerebral hemisphere: Dominant parietal lobe Vascular: Middle cerebral artery

Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: R sided Visual loss – homonymous hemianopia Constructional apraxia - Non-dominant hemisphere Aphasia – receptive - Dominant hemisphere (Wernicke’s area) Anatomy: MCA Inferior Division

Syndrome: Alexia without Agraphia Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Alexia(learning disability characterized by impaired reading) Visual loss – homonymous hemianopia Pure word blindness. Can write but not read. Syndrome: Alexia without Agraphia Anatomy: Cerebral hemisphere: Left occipital region plus Splenium of corpus collosum Vascular: Posterior cerebral artery: Collosal branches

Syndrome: Balint Syndrome Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Both loss of voluntary but not reflex eye movements Both Optic ataxia – poor visual-motor coordination Both Asimultagnosia – inability to understand visual objects Syndrome: Balint Syndrome Anatomy: Cerebral hemisphere: Bilateral parietal-occipital lobes Vascular: Posterior cerebral artery: Bilateral

Syndrome: Claude Syndrome Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Contralateral Ataxia – arm and leg Oculomotor palsy with contralateral tremor and ataxia. Syndrome: Claude Syndrome Anatomy: Midbrain: Tegmentum (Red Nucleus, Reticular Formation, and Substantia Nigra) Vascular: Posterior cerebral artery

Benedikt Syndrome (or Paramedian Midbrain Syndrome): is a midbrain stroke syndrome that involves the fascicles of the oculomotor nerve and the red nucleus resulting in an ipsilateral CN III palsy and crossed hemiataxia and chorea. Claude Syndrome: is one of the brainstem stroke syndromes in which there is infarction of the dorsomedial aspect of the midbrain secondary to occlusion of the small perforating branches of the posterior cerebral artery supplying this area. The infarction involves the medial aspect of red nucleus with the rubrodentate fibers, CN III nucleus and superior cerebellar peduncle. Clinical picture is characteristic and includes ipsilateral oculomotor nerve palsy and contralateral upper and lower limb ataxia. Weber Syndrome: is a midbrain stroke syndrome that involves the fascicles of the oculomotor nerve resulting in an ipsilateral CN III palsy and contralateral hemiplegia or hemiparesis.

Syndrome: Anton Syndrome Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Visual loss – bilateral Unawareness or denial of blindness Syndrome: Anton Syndrome Anatomy: Cerebral hemisphere: Bilateral occipital lobes Vascular: Posterior cerebral artery: Bilateral Basilar artery: Top of the basilar

Syndrome: Lateral Pontine (Marie-Foix Syndrome): Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Ipsilateral Ataxia – arm and leg, Cerebellar tracts Contralateral Weakness – upper and lower extremity, Corticospinal tracts Contralateral Hemisensory loss – pain and temperature, Spinothalamic tract Syndrome: Lateral Pontine (Marie-Foix Syndrome): Anatomy: Lesion in the lateral pons, including the middle cerebellar peduncle. Ipsilateral cerebellar ataxia due to involvement of cerebellar tracts Contralateral hemiparesis due to corticospinal tract involvement Variable contralateral hemi-hypesthesia for pain and temperature due to spinothalamic tract involvement Vascular: Basilar artery: Long circumferential branches Anterior inferior cerebellar artery

Syndrome: Lateral Medullary Syndrome (Wallenberg Syndrome) Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Contralateral Hemisensory loss – pain and temperature Ipsilateral Sensory loss – face, Ataxia, Nystagmus, Nausea/vomiting, Vertigo, Horseness, Dysphagia Ipsilateral Horner syndrome Syndrome: Lateral Medullary Syndrome (Wallenberg Syndrome) Anatomy: Lateral Medulla Vascular: Vertebral artery: Distal branches Vertebral artery: Superior lateral medullary artery Posterior inferior cerebellar artery: Less common than vertebral

Syndrome: Inferior Medial Pontine Syndrome (Foville Syndrome) Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Contralateral Weakness – upper and lower extremity Ipsilateral Weakness – face – entire side Ipsilateral Lateral gaze weakness Syndrome: Inferior Medial Pontine Syndrome (Foville Syndrome) Anatomy: Pons: Unilateral lesion in the dorsal pontine tegmentum in the caudal third of the pons Vascular: Basilar artery: Paramedian branches Basilar artery: Short circumferential arteries

Syndrome: Locked-in Syndrome Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: B/L Weakness – upper and lower extremity, B/L Weakness – face – entire side Lateral gaze weakness Dysarthria Syndrome: Locked-in Syndrome Anatomy: Bilateral ventral pons Vascular: Basilar artery

Syndrome: Medial Medullary Syndrome (Dejerine Syndrome) Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Contralateral Weakness – upper and lower extremity Contralateral Hemisensory loss – vibration and proprioception Ipsilateral, Tongue weakness +/- atrophy Syndrome: Medial Medullary Syndrome (Dejerine Syndrome) Anatomy: Medulla: Medial medulla Vascular: Vertebral artery: Anteromedial artery Anterior spinal artery: Anteromedial artery Basilar artery

Syndrome: Ventral Pontine Syndrome (Raymond Syndrome) Case: 75 yr old R handed male presented with a sudden onset of: Sign & Symptoms: Ipsilateral Lateral gaze weakness – CN VI Contralateral Weakness – upper and lower extremity Syndrome: Ventral Pontine Syndrome (Raymond Syndrome) Anatomy: Pons: Ventral medial pons Vascular: Basilar artery: Paramedian branches If Ipsilateral face weakness – as well then: Millard-Gubler Syndrome (CN VI + VII)

Question: Which trial showed benefit of IV tPA use in the extended window of 3 – 4.5 hours after the symptoms onset? A: NINDS B: ECASS I C: ECASS II D: ECASS II The results of the European Cooperative Acute Stroke Study I (ECASS I), ECASS II and the ATLANTIS trial (Alteplase Thrombolysis for Acute Noninterventional Therapy in Ischaemic Stroke) were negative studies for the use of tPA but used LKW to tPA time of < 6hrs, NINDS showed benefit of tPA within 3hrs, and post-hoc analysis of ECASS II also showed benefit within 3 hours. ECASS III extended the time window to 3 – 4.5 hours.

Question: Of the following stroke risk factors which is the most important and essential to control? A: Hypertension B: Diabetes Mellitus C: Hyperlipidemia D: Smoking Although diabetes, hypertension, smoking, and hyperlipidemia clearly increase stroke risk, hypertension is the most important risk factor, and hypertension control is the most important intervention in this patient to reduce his future risk of cerebral ischemia.

C: Carotid Atheroembolus D: Giant cell arteritis Question: A patient presented to the Urgent Care neurology clinic with complain of diplopia and ptosis. Exam showed A: PCA Aneurysm B: Meningioma C: Carotid Atheroembolus D: Giant cell arteritis This patient has a pupil-sparing third nerve palsy most likely related to diabetes or small brainstem infarct. A posterio communicating artery aneurysm would usually not spare the pupil. Meningioma would often involve cranial nerves 4 and 6 as well and may be associated with proptosis. The third nerve lesion associated with an extrinsic mass typically would not be pupilsparing. It would be unlikely that a carotid atheroembolus or giant cell arteritis would produce a third nerve palsy.

Question: 78 yr old male presented with following imaging: This patient has cerebral amyloid angiopathy, which typically presents in elderly persons with spontaneous lobar hemorrhage. Typically patients have a history of dementia and have evidence of multiple tiny old hemorrhages (best seen on gradient-echo MRI imaging). The other disorders listed are much less likely with the clinical history and findings.

B: Meningitis/Encephalitis C: Subarachnoid hemorrhage Question: 33y old woman h/o pre-eclampsia who delivered a baby at 30wks via c-section on 12/24, was found to have word finding difficulty with right leg stiffness and right arm twitching was brought to the ED and the stroke pager was activated, she had a stiffening of the body with seizure like activity lasting about 3 mins which was aborted by giving 2 mg of ativan and Mg however she continues to have RUE pronator drift. Exam continued to improved and was later back to baseline. A: Pituitary Apoplexy B: Meningitis/Encephalitis C: Subarachnoid hemorrhage D: Venous sinus thrombosis "This patient has cerebral venous sinus thrombosis secondary to postpartum hypercoagulability. Pituitary apoplexy is less likely given the absence of cranial nerve palsies from involvement of the cavernous sinus. Meningitis or encephalitis is unlikely given the absence of fever and meningismus. The patient is not eclamptic, and a subarachnoid hemorrhage from aneurysm would be unlikely in this scenario."

Pearl: Carotid artery dissection may be associated with an ipsilateral Horner syndrome and face or head pain. Pearl: This patient has color agnosia and prosopagnosia most likely due to bilateral ischemia in the mesial inferior occipitotemporal cortex. Visual agnosia occurs due to damage in the association areas of the visual cortex. The lateral occipital complex appears to respond to many different types of objects. Prosopagnosia (inability to recognize faces) is due to damage of the fusiform face area (FFA), in the fusiform gyrus of the temporal lobe. The parahippocampal place area (PPA) of the limbic cortex has been found to be activated by the sight of scenes and backgrounds. Cerebral achromatopsia (the inability to discriminate between different hues) is caused by damage to the V8 area of the visual association cortex. A famous report on this condition is the title essay of Oliver Sacks' book, The Man Who Mistook His Wife for a Hat.

Pearl: Luxury perfusion refers to increased perfusion in the periphery of an ischemic area. It is best documented with PET or SPECT. Some authors have used the term to refer to gray matter enhancement, which is best seen on MRI 3 days to several weeks after a cerebral infarct and results from contrast leaking into the perivascular space in an area of ischemic insult. Pearl: The principle of diffusion MRI is in the microscopic motion of water protons. In acute stroke settings there is a shift of extracellular water into the intracellular space, thereby reducing the apparent diffusion coefficient (ADC).

Question: Which stroke location is significantly associated with increased serum cardiac troponin elevation: A: Thalamus B: Pituitary C: Hypothalamus D: Insular cortex

A: Urgent Interventional Cardiology consult for PFO closure Question: A 45 yr old male presented with sudden onset of R sided weakness with NL fold flattening and gaze preference. He was found to have L MCA stroke and work-up with ECHO showed large PFO. Which of the following is true: A: Urgent Interventional Cardiology consult for PFO closure B: LE Duplex to r/o DVT C: Start patient on anticoagulation D: DO NOTHING E: Hypercoagulable work-up IF patient was found to have DVT on Duplex then how will that change your answer? About 20% of the general population reportedly has a PFO, with 50% of young stroke patients having a PFO. Treatment trial data to date have been inconclusive as to the benefits of PFO closure versus medical management with antithrombotics, except in the setting of deep venous thrombophlebitis or hypercoagulable states. In older patients with PFO the association between thrombophilia and stroke is weak at best; however, if a DVT is present then it would be reasonable to consider treatment with warfarin on the presumption of a paradoxical embolism.

Pearl: The history of migraine or prominent headaches together with stroke-like episodes is strongly suggestive of CADASIL. CADASIL results from a NOTCH3 gene mutation and is inherited as an autosomal dominant trait. The diagnosis in her brother may be incorrect as the relapsing remitting features and MRI appearance of CADASIL may mimic MS. The location of white matter lesions prominently in the anterior temporal regions is strongly suggestive of CADASIL, as is the progressive development of a subcortical dementia. CSF lactate may be elevated in MELAS, an inherited mitochondrial disorder which can present with stroke-like episodes, seizures and headaches. MRI frequently shows patchy lesions in cortical grey matter. Polyglucosan disease is an autosomal recessive disorder of glycogen metabolism associated with upper and lower motor neuron and cognitive changes. There are often prominent MRI white matter changes in the mesencephalon and cerebellum. Findings on sural nerve biopsy are diagnostic. Fabry disease is inherited as an x-linked trait. It can present with stroke, renal insufficiency and cardiomyopathy, and is associated with acroparesthesia, polyneuropathy and angiokeratomas. MRI often shows periventricular white matter changes and leukoaraiosis. Visual evoked potentials can be useful in differentiating MS related optic neuropathy from other leukodystrophies.

Noncontrast axial CT reveals a lens shaped, well localized hyperdense extraaxial lesion with some surrounding edema. Findings are consistent with epidural hematoma. Epidural hematoma is typically a localized hyperdense lesion because dura is adherent to the skull. Acute subdural hematoma is crescent shaped and more widespread. Chronic subdural has low density on CT.

On diffusion-weighted and gradient-echo (T2 On diffusion-weighted and gradient-echo (T2*) sequences there is a right parietal parasagittal intraparenchymal hemorrhage. On T1 pre-contrasted views there is hyperintensity in the superior sagittal sinus, corresponding to thrombosis of this structure. These findings taken together support the diagnosis of a parietal infarct caused by increased venous pressure. Venous infarctions are often hemorrhagic.

The most likely diagnosis is ruptured aneurysm The most likely diagnosis is ruptured aneurysm. The high density of the recent bleeding outlines the subarachnoid space around the brain. Its shape rules out the other types of hemorrhage mentioned as options. There is hydrocephalus, but it is not idiopathic; rather, it is caused by the subarachnoid hemorrhage. The air with pneumocephalus would have a much lower density than any of the structures seen on the image.

The linear high density structure seen on the noncontrast head CT lies at the expected location of the proximal portion of the left middle cerebral artery. This pattern is typical of a middle cerebral artery thrombosis.

The distribution of the changes on the MRI shown is consistent with an acute infarct in the distribution of the anterior cerebral artery. Herpes encephalitis typically involves anterior and medial temporal lobes and inferior frontal lobes.

In the sagittal T1-weighted image, the normal flow void (low signal) is replaced by intraluminal high signal related to thrombosed superior sagittal sinus. The signal intensity of the thrombus over time has the same evolution pattern as intracerebral hematomas.

The uniform filling of this large vascular anomaly and its position identify it as a vein of Galen malformation, which is a congenital anomaly.