A 58-year-old Chilean woman with a 6-year history of anemia and hematuria, diagnosed with Berger's disease and monitored by a nephrologist, with no history of drug use.
One week prior to her medical evaluation, she presented with headache, altered language and a fluctuating lack of strength in the right upper limb.
Neurological examination revealed: a patient was alert, partially oriented, with decreased attention, fluent language with occasional paraphasias, nomination, repetition, understanding and altered writing.
The motor examination revealed a small paresis test with slight pronation of the right upper extremity and indifferent plantar reflex to the right.
The rest of the evaluation was normal.
The patient was hospitalized with the following diagnoses: left silvian acute stroke, Wernicke's aphasia, right hemiparesis, predominantly brachial.
The study was initiated with computed tomography of the brain (CT) and diffusion magnetic resonance imaging (MRI) that showed no ischemic lesions.
A standard electroencephalogram (EEG) showed a left temporal focal slowness theta-delta without epileptiform activity.
Blood tests included normal coagulation tests, severe anemia with hemolytic characteristics and thrombocytopenia.
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The patient was admitted after a few hours of hospitalization with disorientation and psychomotor agitation.
On the second day fever was added up to 39.5 degrees.
In the neurological examination, the patient stood out: a soporous patient, with conjugated gaze diverted to the right, trunk reflexes present.
The nociceptive stimulus mobilized and removed the right hemibody, with left hypotonia and bilateral indifferent plantaris.
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A new CT scan of the brain showed no lesions.
Then, diffusion by MRI showed bilateral hyperintense punctiform images cortico-subcortical, compatible with acute infarctions.
Due to the severe impairment of consciousness, considering a left status epileptiform not coneptvulsive, a second standard EEG was performed, which showed disorganization of the basal activity, with marked decrease in voltage in the right and right derivations.
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In the laboratory bilirubin was detected from hemolysis indicators with increased indirect hemolysis and LHD, as well as increased thrombocytopenia and creatinine.
A diagnosis of TTP was made and treatment with plasmapheresis and daily fresh frozen plasma transfusions was initiated.
Subsequently, the activity of fVW metalloproteinase was measured, which was <5%. The study of cancer and mesenchymopathies was negative.
The patient was admitted with episodes of generalized tonic-clonic seizures and phenytoin was added.
On the fifth day of treatment, there was partial recovery of neurological impairment with spontaneous wakefulness, poor language, non-affected, mobilization of the right hemibody and hypotonia and left brachiocrural paresis, with extensor plantar response.
On the sixth day of treatment, the patient developed fever and hemodynamic compromise, with sepsis of pulmonary focus, septic shock and severe respiratory failure were diagnosed, requiring invasive mechanical ventilation, use of vasoactive drugs and antibiotic therapy.
Due to the need for sedation, posterior neurological assessment was performed but left hemibody hypotonia persisted.
The patient died of multiorgan failure at 15 days of hospitalization and 13 of treatment with PF and transfusion of PFC, receiving three boluses of methylprednisolone.
