Shunt Nephritis: An Increasingly Unfamiliar Diagnosis.

BACKGROUND: Shunt nephritis is a rare, reversible immune-complex mediated complication of cerebrospinal fluid (CSF) shunt infection that can progress to end-stage renal disease and even death if diagnosis is delayed. CASE DESCRIPTION: The present case report details the manifestation and clinical course of shunt nephritis in a 50-year-old patient who presented with symptoms of nephrotic syndrome 30 years after ventriculojugular shunt placement. Diagnosis was delayed due to initial negative CSF and blood cultures, but a later CSF culture was positive for Propionibacterium acnes. After treatment with intravenous antibiotics and complete removal of shunt with subsequent replacement with a new ventriculoperitoneal shunt, the nephritic symptoms resolved, but the patient continued to have reduced kidney function consistent with stage IIIa chronic kidney disease. CONCLUSION: This case emphasizes the clinical importance of having a high index of suspicion in patients with a ventricular shunt who present with symptoms consistent with nephritis, even in the setting of negative cultures and delayed presentation.

High signal in the cerebrospinal fluid following prior gadolinium administration in a patient with renal impairment.

Increased signal intensity in the cerebrospinal fluid (CSF) on magnetic resonance imaging due to the presence of gadolinium is rarely observed, but has been seen in patients with brain or spinal pathology or underlying renal impairment. We report this phenomenon in a 66-year-old woman with diabetic nephropathy and discuss the possible pathogenesis of the scan findings. Recognition of this unusual finding, and features distinguishing it from other causes of high CSF signal intensity, such as subarachnoid haemorrhage and protein in the CSF, are emphasised to help prevent diagnostic errors.

Contrast enhancement of cerebrospinal fluid on delayed MRI in a patient with an epidural abscess and renal failure.

Cerebrospinal fluid (CSF) is normally of low signal intensity on T1 weighted MRI. The case of a patient with renal failure who developed an epidural abscess, and in whom the CSF appeared of high signal intensity on delayed T1 weighted MRI is presented. Possible mechanisms for this are discussed.

Persistence of gadolinium in CSF: a diagnostic pitfall in patients with end-stage renal disease.

Two dialysis-dependent patients with end-stage renal disease underwent brain and spine MR imaging a few days after having undergone gadolinium-enhanced imaging studies. Increased signal intensity in the subarachnoid space on T1-weighted and fluid-attenuated inversion recovery images was noted. Excretion of gadolinium into the CSF was proven in one case by mass spectrometry. Dialysis-dependent patients with end-stage renal disease and neurologic abnormalities often undergo contrast-enhanced MR imaging. Recognition that these patients may show increased signal intensity in the subarachnoid space because of gadolinium excretion into CSF may prevent diagnostic errors.

Gadolinium enhancement of cerebrospinal fluid in a patient with renal failure.

Gadolinium based MRI contrast agents are considered very safe due to their well known pharmacologic properties and elimination mechanisms. In this paper, we present a unique case in whom transient enhancement of CSF with contrast is seen. Severe renal failure is demonstrated to be responsible for this finding. The diagnostic criteria for everyday clinical setting and possible clinical implications are discussed.

Cerebrospinal fluid levels of amino acids in infants and young children with chronic renal failure.

Chronic renal failure (CRF) is associated, especially in young children, with delayed cognitive development of unknown origin. As cerebrospinal fluid (CSF) reflects the composition of the extracellular fluid of the brain, not only plasma but also CSF amino acids concentrations were determined in 8 infants (age 2-8 months) and 3 children (age 26, 32 and 56 months) with CRF (creatinine clearance 13 +/- 9 ml/min/ 1.73 m2). In three of these children investigations were repeated after six weeks of CAPD treatment. In the infants, a significant decrease was found in CSF of alpha-aminobutyric acid, valine, isoleucine, leucine, tyrosine, tryptophane, histidine and n-zeta-methyl-1-lysine, whereas there was a significant increase of 3-methylhistidine. In plasma serine, valine, leucine, tyrosine and histidine were significantly decreased, whereas there was a significant increase of aspartic acid, citrulline, and 3-methylhistidine. These abnormalities remained constant after the start of CAPD except for the normalization in CSF and plasma of 3-methylhistidine. These data indicate a generalized disturbance of amino acids in young children with CRF. An abnormal substrate is offered to the neurons and astroglia in children with CRF.

Famotidine-associated central nervous system reactions and plasma and cerebrospinal drug concentrations in neurosurgical patients with renal failure.

Central nervous system toxicity of H2-receptor antagonists has rarely been confirmed by the respective elevated cerebrospinal fluid drug concentrations. We observed two hemodialyzed neurosurgical patients in whom mental deterioration and convulsions developed after intravenous famotidine therapy (10 and 40 mg/day). Their cerebrospinal fluid drug concentrations were grossly elevated (i.e., 160 and 249 ng/ml) compared with those obtained from three other hemodialyzed neurosurgical patients who exhibited no central nervous system reactions (i.e., 47 to 85 ng/ml). In addition, the mean cerebrospinal fluid/plasma drug concentration ratio obtained from these five neurosurgical patients with renal failure (i.e., 0.46) and that from 10 other neurosurgical patients with normal renal function (i.e., 0.41) were about four times greater than that previously reported from non-neurosurgical patients with normal renal function (i.e., 0.12). Our observation suggests that patients with not only renal dysfunction but also following neurosurgical operations have an excessive accumulation of famotidine in the central nervous system and are more susceptible to the drug-induced adverse central nervous system reactions.

Chromogranin A immunoreactivity in human cerebrospinal fluid: properties, relationship to noradrenergic neuronal activity, and variation in neurologic disease.

Although measurement of chromogranin A in the bloodstream is of value in sympathoadrenal investigations, little is systematically known about chromogranin A in cerebrospinal fluid, despite substantial knowledge about its occurrence and distribution in brain. We therefore applied a homologous human chromogranin A radioimmunoassay to cerebrospinal fluid, in order to evaluate the properties and stability of cerebrospinal fluid chomogranin A, as well as its relationship to central noradrenergic neuronal activity, to peripheral (plasma) chromogranin A, and to disease states such as hypertension, renal failure and Parkinsonism. Authentic, physically stable chromogranin A immunoreactivity was found in cerebrospinal fluid (at 37-146 ng/ml; mean, 87.0 +/- 6.0 ng/ml in healthy subjects), and several lines of evidence (including 3.39 +/- 0.27-fold higher chromogranin A in cerebrospinal fluid than in plasma) indicated that it originated from a local central nervous system source, rather than the periphery. Cerebrospinal fluid chromogranin A values were not influenced by administration of effective antihypertensive doses of clonidine or propranolol, and were not related to the cerebrospinal fluid concentrations of norepinephrine, methoxyhydroxyphenylglycol, or dopamine-beta-hydroxylase; thus, cerebrospinal fluid chromogranin A was not closely linked to biochemical or pharmacologic indices of central noradrenergic neuronal activity. Cerebrospinal fluid chromogranin A was not changed (P > 0.1) in essential hypertension (84.2 +/- 14.0 ng/ml) or renal failure (72.2 +/- 13.4 ng/ml), despite a marked (7.1-fold; P < 0.001) increase in plasma chromogranin A in renal failure, and a modest (1.5-fold; P = 0.004) increase in plasma chromogranin A in essential hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

Accelerated neuropathy of renal failure.

OBJECTIVE: To describe a syndrome of rapidly evolving polyneuropathy in patients with severe renal failure. DESIGN: Retrospective case series of four patients. SETTING: In-hospital evaluations and personal examinations of patients. PATIENTS: Four patients with severe or end-stage renal failure who were receiving peritoneal dialysis. RESULTS: These patients had an acute or subacute syndrome characterized by generalized limb weakness over days or weeks, severe imbalance, diminished reflexes, and numbness. Spinal fluid protein levels were elevated and some demyelinating features were noted on electrophysiological testing. Improvement occurred with more frequent peritoneal dialysis in one patient and transplantation in another, but the neuropathy progressed in the other two in whom diabetes may have played a role. CONCLUSION: This partly reversible acute uremic neuropathy, which is probably caused by the metabolic disturbances of end-stage renal failure, simulates Guillain-Barré syndrome or chronic inflammatory demyelinating polyneuropathy and may be complicated by diabetic neuropathy.

Cerebrospinal fluid amino acids, purines and pyrimidines as a tool in the study of metabolic brain diseases.

After establishing more extended reference values for amino acids, purines and pyrimidines in cerebrospinal fluid (CSF) in infancy and childhood, we studied 1250 CSF-aliquots from patients who were undergoing a diagnostic lumbar puncture for diverse clinical indications. Our primary aim was to answer the question whether determination of the concentration of amino acids, purines and pyrimidines in CSF is a useful tool in screening for metabolic disorders in children with unexplained mental retardation. In unexplained mental retardation (95 patients) we observed varying abnormalities of CSF. These were reproducible in only 2 patients (a decrease of homocarnosine in combination with two unidentified compounds). Striking abnormalities in pyrimidine content which are limited to CSF are found in argininosuccinic aciduria and uraemia. In uraemia a general decrease in amino acids in CSF and increase of gamma-aminobutyric acid (GABA) was observed. The results obtained indicate that determination of amino acids, purines and pyrimidines in CSF is only of limited value in the diagnosis of unexplained mental retardation.

Disturbances of cerebral purine and pyrimidine metabolism in young children with chronic renal failure.

Chronic renal failure during childhood may be associated with delayed cognitive development. From 10 children with chronic renal failure, aged 2-59 months, plasma and cerebrospinal fluid (CSF) purines and pyrimidines have been determined. A marked increase of pseudouridine and cytidine was demonstrated in CSF of 10 and 8 children, respectively. The plasma concentration of pseudouridine was increased in a varying degree to a maximal value of more than 10 times the upper limit of normal. The plasma concentration of cytidine showed only moderately elevated values. In 3 children the study of CSF and plasma was repeated 6 weeks after the start of continuous ambulatory peritoneal dialysis. The abnormal concentrations of pseudouridine and cytidine were still present in CSF and plasma. Further studies are necessary to elucidate the cause of this unknown biochemical aberration of the central nervous system.

Guanidino compounds in serum and cerebrospinal fluid of non-dialyzed patients with renal insufficiency.

Twelve guanidino compounds were determined in simultaneously sampled serum and cerebrospinal fluid of eight non-dialyzed patients with renal insufficiency. Liquid cation exchange chromatography with a highly sensitive fluorescence detection method was used. In patients with serum urea levels about 10 times higher than in controls, the levels of guanidinosuccinic acid, creatinine, guanidine and methylguanidine, in serum as well as in cerebrospinal fluid, are at least 10 times higher than in control subjects. The levels of argininic acid and N-alpha-acetylarginine (in serum) and gamma-guanidinobutyric acid (in cerebrospinal fluid) are slightly increased (less than 10 X). The levels of the other guanidino compounds are close to normal values. A significant positive correlation exists between the guanidinosuccinic acid, creatinine and guanidine levels in serum and cerebrospinal fluid. The accumulation of several experimentally proven toxic guanidino compounds could contribute to the complex nervous system symptomatology and the hematological complications seen in renal insufficiency.

Interrelations between cerebrospinal fluid and plasma inorganic ions and glucose in patients with chronic renal failure.

The concentrations of inorganic ions and glucose in the plasma and CSF of 11 patients with "steady-state" chronic renal failure have been measured and their CSF: plasma interrelations studied. The results have been compared with the corresponding data from 34 control subjects. In the patients with renal failure, there was a positive correlation between raised CSF and plasma potassium concentrations. In contrast to the impaired potassium homeostasis, normal CSF magnesium and calcium concentrations were observed despite wide variations in the plasma concentrations of these ions.

Studies of cerebrospinal fluid and plasma amino acids in patients with steady-state chronic renal failure.

The concentration of free amino acids in the plasma and lumbar CSF of 11 patients with steady-state chronic renal failure has been measured and the CSF: plasma concentration ratios calculated. The results have been compared with the corresponding data from 37 control subjects. In renal failure, elevation of the mean plasma concentration of total amino acids and a reduction in the ratio of essential to total amino acids have been found. Whereas some individual plasma amino acid concentrations in renal failure were higher than normal, others were lower. Striking abnormalities of the CSF amino acid concentration have been observed. Some amino acids have shown similar patterns of abnormality in both CSF and plasma, whereas in the case of others, the changes have been restricted to either CSF or plasma. Significant variations from normal of the CSF: plasma concentration ratios were observed for four amino acids.

The effect of haemodialysis on cerebrospinal fluid and plasma amino acids.

The concentration of free amino acids in CSF and plasma has been measured in 19 patients with renal disease undergoing haemodialysis therapy. The values obtained have been compared with those measured in non-haemodialysed patients. At the end of a 10-h dialysis period, the total CSF amino acid concentration had fallen by only 23.3% compared with a 57.7% fall in plasma. Examination of the results for individual amino acid concentrations showed 3 different patterns of inter-relationship between plasma and CSF.

Polyols in the cerebrospinal fluid and plasma of neurological, diabetic and uraemic patients.

Five polyols, arabinitol, anhydroglucitol, mannitol, sorbitol and myoinositol, normally present in the cerebrospinal fluid (CSF), were studied. Quantitative gas-liquid chromatographic analysis of 211 CSF and 112 plasma samples indicated significantly altered concentrations in several clinical conditions. All five polyols were decreased in the CSF of patients suffering from meningitis, cerebral atrophy, sepsis, and in patients receiving intrathecal cytostate therapy. Equilibration between plasma and CSF may explain the changes in sepsis and meningitis, while decreased total number of functioning cells may cause the decrease in cerebral atrophy. Intrathecal cytostates seem to have a destroying effect on the cell metabolism of the central nervous system. Renal failure causes accumulation of polyols in the plasma. Alterations in the metabolism of sorbitol, myoinositol and anhydroglucitol seem to be present in diabetes. The plasma concentration of anhydroglucitol is decreased in renal failure.

Plasma, red cell and cerebrospinal fluid concentrations of mannitol and sorbital in patients with severe chronic renal failure.

The concentrations of mannitol and sorbitol in plasma, red cells, cerebrospinal fluid and urine were determined in 24 patients with chronic renal failure; 10 of them were on conservative treatment and 14 were haemodialysed three times weekly. The mannitol concentration was significantly increased in the plasma and the cerebrospinal fluid of the uraemic patients compared with the controls. In six out of ten uraemic patients mannitol clearance values exceeded creatinine clearance values. The plasma concentration of sorbitol was undetectable or very low in all patients and control subjects. Red-cell and cerebrospinal fluid concentrations of sorbitol showed a large individual variation in the uraemic patients on conservative treatment and did not correlate with kidney function. During dialysis the mannitol concentration decreased, leading to a small osmotic gradient between the plasma and the red cells. The changes in the concentration of mannitol during dialysis showed no connection with the symptoms of central nervous disturbance which appeared during dialysis treatment. The red cell sorbitol concentration during dialysis increased by about 20%. There was a correlation (p less than 0.05) between t,e increase in sorbital in cereemic patients.

Plasma, red cell and cerebrospinal fluid concentrations of myoinositol in patients with severe chronic renal failure.

A study was made of 24 patients with severe chronic renal failure; 14 of them were undergoing regular haemodialysis treatment 3 times weekly. Plasma, red-cell and cerebrospinal fluid concentrations of myoinositol were increased in all the patients treated conservatively. The plasma level of myoinositol correlated with the plasma level of creatinine (r = 0.78). The plasma myoinositol level increased more than the CSF and red-cell levels, indicating that the myoinositol in the red cells and the CSF originated mostly in plasma. Dialysis for eight hours produced a fall of about 50% in the level of myoinositol in plasma while the decrease in red-cell myoinositol was negligible. This lead to an osmotic grandient between extra- and intra-cellular myoinositol which was however small in molar terms and did not correlate with symptoms of central neurological disturbances.