28033278 Visualization

The 36 Age - year Age - old Age male Sex patient in this case had a 6 Duration - year Duration history of diffuse Detailed_description cutaneous Biological_structure SSc Disease_disorder (Fig.1) and had not received regular follow Clinical_event - up Clinical_event or medication Medication therapy.
He had experienced malaise Sign_symptom , poor Sign_symptom appetite Sign_symptom , and progressive Detailed_description shortness Sign_symptom of Sign_symptom breath Sign_symptom for 1 Duration month Duration and oligouria Sign_symptom for 3 Duration days Duration .
He was admitted Clinical_event to our hospital Nonbiological_location with renal Disease_disorder failure Disease_disorder and pulmonary Biological_structure edema Sign_symptom .
In the emergency department, his blood Diagnostic_procedure pressure Diagnostic_procedure , pulse Diagnostic_procedure rate Diagnostic_procedure , and respiratory Diagnostic_procedure rate Diagnostic_procedure were 174/127 Lab_value mm Lab_value Hg Lab_value , 88/min Lab_value , and 22/min Lab_value , respectively.
Laboratory Diagnostic_procedure analysis Diagnostic_procedure revealed the following values: white Diagnostic_procedure blood Diagnostic_procedure cell Diagnostic_procedure count Diagnostic_procedure , 7730/μL Lab_value (3500–11,000/μL); hemoglobin Diagnostic_procedure , 8.6 Lab_value   g/dL Lab_value (12–16 g/dL): platelet Diagnostic_procedure count Diagnostic_procedure , 87000/μL Lab_value (150,000–400,000/μL); blood Diagnostic_procedure urea Diagnostic_procedure nitrogen Diagnostic_procedure , 78 Lab_value   mg/dL Lab_value (6–21 mg/dL); creatinine Diagnostic_procedure , 8.9 Lab_value   mg/dL Lab_value (1.1–1.5 mg/dL); calcium Diagnostic_procedure , 8.2 Lab_value   mg/dL Lab_value (8.8–10.3 mg/dL); phosphorus Diagnostic_procedure , 5.4 Lab_value   mg/dL Lab_value (2.7–4.5 mg/dL); haptoglobin Diagnostic_procedure , <6.56 Lab_value   mg/dL Lab_value (30–200 mg/dL); and lactate Diagnostic_procedure dehydrogenase Diagnostic_procedure , 547 Lab_value   U/L Lab_value (106–211 U/L).
A routine urinalysis Diagnostic_procedure revealed a proteinuria Lab_value score Lab_value of Lab_value 2+ Lab_value ( 200 Lab_value   mg/dL Lab_value ), white Diagnostic_procedure blood Diagnostic_procedure cell Diagnostic_procedure count Diagnostic_procedure of 3–5/high power Lab_value field Lab_value , and red Diagnostic_procedure blood Diagnostic_procedure cell Diagnostic_procedure count Diagnostic_procedure of 25–50/high power Lab_value field Lab_value .
The autoimmune Diagnostic_procedure profile Diagnostic_procedure indicated an antinuclear Diagnostic_procedure antibody Diagnostic_procedure level of 1:320 Lab_value ( speckles; Lab_value normal Lab_value , <1:40 Lab_value ).
Tests for anti Diagnostic_procedure - Scl70 Diagnostic_procedure , anti Diagnostic_procedure - double Diagnostic_procedure - stranded Diagnostic_procedure DNA Diagnostic_procedure , anti Diagnostic_procedure - Ro Diagnostic_procedure , anti Diagnostic_procedure - La Diagnostic_procedure , and anticardiolipin Diagnostic_procedure antibodies Diagnostic_procedure were all negative Lab_value .
Kidney Biological_structure echogram Diagnostic_procedure showed a decreased Sign_symptom bilateral Sign_symptom kidney Sign_symptom size Sign_symptom without hydronephrosis Sign_symptom .
Despite the chronic changes visible on the echogram, the patient had developed acute pulmonary Biological_structure edema Sign_symptom and oligouria Sign_symptom only 3 Date days Date before Date admission.
Accordingly, a clinical diagnosis of acute Detailed_description - on Detailed_description - chronic Detailed_description renal Disease_disorder failure Disease_disorder was made.
A further diagnosis of SRC Disease_disorder was supported by the presence of renal Disease_disorder failure Disease_disorder with microangiopathic Detailed_description hemolytic Detailed_description anemia Sign_symptom and hypertension Sign_symptom , and the patient was started on captopril Medication therapy.
The sustained deterioration Sign_symptom in Sign_symptom renal Sign_symptom function Sign_symptom and anuria Sign_symptom had led to a requirement for regular Detailed_description hemodialysis Therapeutic_procedure from admission.
After Date 3 Date days Date , captopril Medication was changed to amlodipine Medication because the patient developed a severe Severity , intolerable Detailed_description cough Sign_symptom thought to be associated with captopril.
His systolic Diagnostic_procedure blood Diagnostic_procedure pressure Diagnostic_procedure was controlled Lab_value between Lab_value 140 Lab_value and Lab_value 180 Lab_value mm Lab_value Hg Lab_value .
At approximately 3 Date weeks Date after Date the initiation of maintenance hemodialysis Coreference , the patient newly developed a generalized Detailed_description tonic Detailed_description - clonic Detailed_description seizure Disease_disorder disorder Disease_disorder .
A brain Biological_structure computed Diagnostic_procedure tomography Diagnostic_procedure ( CT Diagnostic_procedure ) scan revealed a small Severity lacunar Detailed_description infarct Disease_disorder over the left Biological_structure basal Biological_structure ganglion Biological_structure without intracranial Biological_structure hemorrhage Disease_disorder or large Severity infarct Disease_disorder .
The seizure Sign_symptom resolved spontaneously without anticonvulsants Medication , and the patient was finally discharged Clinical_event home Nonbiological_location without incident and scheduled for regular hemodialysis Therapeutic_procedure thrice Frequency weekly Frequency .
However, at 1 Date week Date after Date discharge, the patient developed a sudden Detailed_description - onset Detailed_description headache Sign_symptom and vomiting Sign_symptom with confusion Sign_symptom and recurrence of the generalized Detailed_description tonic Detailed_description - clonic Detailed_description seizure Sign_symptom .
He presented Clinical_event at the emergency Nonbiological_location department Nonbiological_location with a blood Diagnostic_procedure pressure Diagnostic_procedure of 183/100 Lab_value mm Lab_value Hg Lab_value , platelet Diagnostic_procedure count Diagnostic_procedure of 149,000/μL Lab_value , serum Diagnostic_procedure LDH Diagnostic_procedure of 332 Lab_value   U/L Lab_value , and a peripheral Diagnostic_procedure blood Diagnostic_procedure smear Diagnostic_procedure containing Lab_value 2–3  schizocytes/HPF Lab_value (Fig.2).
Brain Biological_structure CT Diagnostic_procedure revealed no Lab_value interval Lab_value change Lab_value , and lumbar Diagnostic_procedure puncture Diagnostic_procedure revealed traumatic Lab_value tapping Lab_value only Lab_value .
Cerebrospinal Biological_structure fluid Diagnostic_procedure cultures Diagnostic_procedure were negative Lab_value for bacteria Detailed_description , mycobacteria Detailed_description , and viruses Detailed_description .
Brain Biological_structure magnetic Diagnostic_procedure resonance Diagnostic_procedure imaging Diagnostic_procedure ( MRI Diagnostic_procedure ) showed bilateral Detailed_description hyperintensity Sign_symptom in the occipital Biological_structure and Biological_structure parietal Biological_structure lobes Biological_structure on a fluid Detailed_description - attenuated Detailed_description inversion Detailed_description recovery Detailed_description (FLAIR) Detailed_description sequence Detailed_description (Fig.3).
Finally, the patient was diagnosed with PRES Disease_disorder .
We resumed captopril Medication therapy at a dose of 25 Dosage   mg Dosage thrice Dosage daily Dosage .
As it was difficult to differentiate SRC from TTP, we also considered plasma exchange treatment.
However, the patient's mental Diagnostic_procedure status Diagnostic_procedure returned to normal Lab_value within 3 Date days Date in the absence of plasma Therapeutic_procedure exchange Therapeutic_procedure , and a follow-up MRI Diagnostic_procedure of the brain Biological_structure 2 Date months Date later Date showed complete resolution of cerebral Biological_structure edema Sign_symptom (Fig.4).
Therefore, SRC Detailed_description - related Detailed_description PRES Disease_disorder was confirmed.
Although the patient experienced a full Sign_symptom neurologic Sign_symptom recovery Sign_symptom , his renal Diagnostic_procedure function Diagnostic_procedure did Lab_value not Lab_value improve Lab_value and he remained dialysis Therapeutic_procedure dependent Detailed_description .