When Kidneys and Brain Cry

History and Physical

A 3-year-old girl has had bloody diarrhea for 4 days. On physical examination, the child was afebrile and had normal blood pressure but looked pale and dehydrated. Her skin showed petechial hemorrhages on the buttocks and legs. She was irritable with a fluctuating mental status examination due to lethargy. The rest of the neurologic examination was unremarkable. Hemoglobin was 8.7 g/dl, hematocrit was 29%, and the white blood cell (WBC) count was 39,000/ul, with a marked shift to the left. Blood urea nitrogen was 91 mg/dl, creatinine was 3.6 mg/dl, sodium was 120 mEq/l, and potassium was 4.7 mEq/l. The next day, the hematocrit decreased to 19.7%, and she was transfused with RBCs, after which her level of consciousness improved, but she became oliguric and hypertensive (130/90 mm Hg), so hemodialysis was initiated. She continued to be oliguric and hypertensive, and 8 days after admission she became unresponsive with gasping respirations and developed generalized tonic-clonic seizures. She was intubated and received peritoneal dialysis and anticonvulsant therapy.

Diagnostic Workup

Initial head CT showed bilateral and asymmetric hypodense lesions in the lentiform nuclei and thalami ( Fig. 75.1 ). EEG showed intermittent epileptiform activity without associated motor seizures.

An axial computed tomography scan with arrows marking low-density areas in the lenticular nuclei and anterior thalami. The axial computed tomography scan presents a narrow midline third ventricle flanked by symmetric lateral ventricles. Two small arrow pointers mark round areas of reduced density at the sites of the lenticular nuclei beside the internal capsules. Two larger arrow pointers mark oval areas of reduced density at the front edges of each thalamus, adjacent to the third ventricle. A uniform band of cerebral cortex outlines the periphery of the slice. — Fig. 75.1

Brain MRI on the 14th day after admission showed hemorrhagic necrosis in the bilateral basal ganglia and surrounding white matter ( Fig. 75.2 ).

Four axial scan panels labeled A to D with thin arrows marking bright bilateral regions in the lenticular nuclei and arrowheads marking centers. The four scan panels are labeled A to D. Panel A presents an axial fluid attenuated inversion recovery magnetic resonance imaging scan labeled A. Thin arrows indicate bilateral bright regions at the posterior lenticular nuclei that extend into the internal, external, and extreme capsules. Triangular arrowheads appear within the central portions of those bright regions. Panel B presents an axial T 1-weighted magnetic resonance imaging scan labeled B. Thin arrows indicate bilateral bright regions at the posterior lenticular nuclei that extend into the internal, external, and extreme capsules. Triangular arrowheads appear within the central portions of those bright regions. Panel C presents an axial diffusion-weighted imaging scan labeled C. Thin arrows indicate bilateral areas of high signal intensity at the posterior lenticular nuclei. A triangular arrowhead appears within the central portion of the right-sided area. Panel D presents an axial apparent diffusion coefficient map labeled D. Thin arrows indicate bilateral areas of low signal intensity at the posterior lenticular nuclei. A triangular arrowhead appears within the central portion of the left-sided area. — Fig. 75.2

The child’s renal and neurologic status gradually stabilized, and she was extubated 15 days after admission.

Clinical Differential Diagnoses

Infectious diseases such as shigellosis or meningococcemia can present with seizures with or without fever, renal failure, and skin petechiae.

Posterior reversible encephalopathy syndrome (PRES) presents with seizures, visual deficits, and alteration of consciousness that can be associated with renal failure (acute glomerulonephritis, lupus nephritis) ( Fig. 75.3 ).