A 15-year-old chilean woman with a history of vascular type Ehlers-Danlos syndrome diagnosed by genetic study at 8 years of age. She consulted for a 5-day history of paresthesia left upper limb paresthesia hollow.
She did not present consciousness compromise or seizures.
He reported that the symptoms appeared spontaneously without apparent cause.
General physical appearance: ecchymal and translucent skin with multiple ecchymosis predominantly in the upper limbs.
neurological impairment: vigil, time-oriented spatially, normal visual field, expression aphasia, right labial commissure deviation, left phciobrachial-crural hemiparesis with motility2.
plantar foot extending to the left.
No sensory deficit.
No meningeal signs.
Laboratory tests: parameters within normal range.
Cerebral non-contrast computerized axial tomography (CAT) showed a right subcortical infarction and was hospitalized for study and treatment, starting neuroprotective therapy.
On the second day, a contrast-enhanced brain scan showed a right subacute sylvian infarction that compromised deep and superficial territories, and a small intracranial hemorrhage later performed a right radiated crown CAT scan of 9 mm.
Due to the history of Ehlers-Danlos syndrome type IV, it was decided to complete a study with magnetic resonance imaging of the brain encephalon complemented with magnetic angioresonance with contrast and angioresonance of the right frontal nucleus, which also involved an ipsilateral frontal lobe, with a cerebral infarct of the right-brain
There was no evidence of hemorrhagic transformation or mass effect.
In a polygonal study, the lack of representation of the right internal carotid artery stood out, which, as demonstrated in the study of neck vessels, presented a dissection close to its origin.
The right anterior and middle cerebral arteries were reconstructed through the anterior communicating artery.
In an perfusion study, a significant mist was evidenced.
As a variant of aortic arch, the left subclavian artery was born distal to the right subclavian artery.
Study challenge without significant findings.
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Considering the presence of mismatch, where the risk tissue was greater than the infarcted one, it was decided that the most important action was to improve mean arterial pressure (MAP) and maintain it between 80-90 mmHg in order to optimize the first few days.
She presented an episode of disconnection associated with sphincter relaxation. An electroencephalogram was requested, which was altered by the presence of theta intermittent delta delta delta hyper32 temporal center stimulation photoventilation channels, drowsiness.
Neurology team ruled out absence seizures and proposed that anticonvulsant therapy with lamotrigine should be started in the event of a new seizure episode, under suspicion of complex partial seizures.
The control brain CT scan showed no evolutionary changes.
Over the course of the days, the patient developed aphasia, with mild dysarthria and left brachiocrural M4 hemiparesis, without other neurological complications.
Regarding management, considering complete arterial occlusion and risk of spontaneous gastrointestinal and other territory bleeding, anticoagulant treatment was not indicated and maintained with cautious platelet antiaggregation (5 days with acetylsalicylic acid 250 mg/day).
Due to his good evolution, he was discharged with control in the polyclinic of physiatry, neurology and vascular surgery.
One month later, the neurologist discussed the case and stated that they maintained acetylsalicylic acid and added proloxetine (10 mg every 12 h).
Vascular clamp agreed to maintain platelet antiaggregation and beta block.
Five months after discharge, magnetic resonance angiography showed reconstitution of the right internal carotid artery, confirming arterial recanalization.
