Journal of Postgraduate Medicine Medknow Publications and Staff Society of Seth GS Medical College and KEM Hospital, Mumbai, India ISSN: 0022-3859 EISSN: 0972-2823 Vol. 46, Num. 3, 2000, pp. 184-186

Journal of Postgraduate Medicine, Vol. 46, No. 3, July-September, 2000, pp. 184-186

Case Report

Nephritis and Cerebellar Ataxia: Rare Presenting Features of Enteric Fever

Parmar RC, Bavdekar SB, Houilgol R, Muranjan MN

Department of Paediatrics, Seth G. S. Medical College & K. E. M. Hospital, Parel, Mumbai - 400 012, India
Address for correspondence: Ramesh Parmar, MD, 2/11, Ambika Nivas, Prabhat Colony, Santacruz (East), Mumbai - 400 055, India E-mail: ramehsparmar@yahoo.com

Code Number: jp00066

Abstract

Enteric fever is a common infectious disease of the tropical world, about 80% of these cases occur in Asian countries. Enteric fever presenting with isolated cerebellar ataxia or nephritis is rare. We report three cases of enteric fever that presented with these complications. Isolated cerebellar ataxia usually occurs in the second week, whereas in our cases it presented within first four days of fever. The common complications of enteric fever related to the urinary tract are cystitis, pyelitis, and pyelonephritis. Glomerulonephritis is uncommon. Most patients with enteric glomerulonephritis present with acute renal failure, hypertensive encephalopathy, or nephritic syndrome. In comparison, our case had milder manifestations. All three patients were treated with parenteral ceftriaxone and showed a prompt recovery. (J Postgrad Med 2000; 46:184-186)

Key Words: Enteric fever, cerebellum, ataxia, renal failure, glomerulonephritis.

Enteric fever is a common infectious disease of the tropical world with an annual worldwide incidence of six million cases and 600,000 deaths. Almost 80% of these cases occur in Asian countries.¹ With the advent of newer and effective antibiotics, the disease usually runs a benign course with complications occurring only in a miniscule minority. Shock and encephalopathy are the most common complications. Enteric fever presenting with isolated cerebellar ataxia or nephritis is rare.^2,3 We report three cases of enteric fever with these complications.

Case Reports

Case 1:

An eleven-years-old girl presented with intermittent fever of 7 days' duration with unsteadiness of gait and difficulty in speech for 3 days. There was no history suggestive of a particular focus of infection, drug ingestion, otorrhoea, trauma, or exanthematous illness. Neurological examination revealed presence of cerebellar signs in the form of dysarthria, past- pointing, broad- based gait, intentional tremors, dysdiadochokinesia and positive tandem walking test. Her abdominal examination revealed a soft, non-tender liver that was palpable 2 cm below the right costal margin (span 6 cm) and a non-tender, soft spleen that was palpable 2 cm below the left costal margin. No other abnormalities were detected on general or systemic examination. Investigations revealed a leucocyte count of 5000 per mm³ (polymorphs 80%, lymphocytes 20%) and blood culture grew Salmonella typhi. Magnetic resonance imaging of the brain was normal. She was treated with ceftriaxone for 14 days. Her ataxia improved progressively and clinical abnormalities disappeared by the 9^th day of treatment. She made an uneventful recovery.

Case 2:

A six-years-old girl presented with fever for nine days and unsteadiness of gait for four days. There was no history of trauma, otorrhoea, drug ingestion, or exanthematous illness. Her neurological examination revealed broad-based gait, cerebellar signs in the form of intentional tremors, past pointing, dysdiadochokinesia and dysarthria. Per abdominal examination revealed a soft, non-tender liver that was palpable 2 cm below the right costal margin (span 8 cm) and a soft, non-tender spleen that was palpable 2.5 cm below the left costal margin. Rest of the general and systemic examination was normal. Total leucocyte count was 6,200 (45% polymorphs, 52% lymphocytes, 3% monocytes) and Widal test was positive (Salmonella typhi `O' and `H' titres of 1:240 each). Blood culture revealed a growth of Salmonella typhi. The patient was treated with parenteral ceftriaxone for 10 days. Her fever disappeared within three days; ataxia and cerebellar signs improved progressively and disappeared at the end of six days.

Case 3:

A 25-months-old girl presented with fever since 15 days, haematuria for 7 days and puffiness of face for 5 days. There was no history of oliguria, dysuria, or abdominal pain. She had not suffered from pyoderma in the recent past, nor was there a history of similar episode previously. Her examination revealed a sick looking, febrile (axillary temperature 38.6°C), normotensive (BP 104/70 mm Hg) child with puffiness of face and oedema feet. Per abdomen examination revealed a soft non-tender enlarged liver with smooth surface (span 8 cm) that was palpable 3 cm below the right costal margin. The spleen was firm, non-tender and was palpable 2 cm below the left costal margin. Abdominal wall oedema and vulval oedema were present. There was evidence of free fluid in the abdomen, too. The rest of the clinical examination was non-contributory. Investigations revealed haemoglobin concentration of 7.6 gm%, total leucocyte count of 14,200 per mm³ (polymorphs 64%, lymphocytes 34%, basophils 2%) and erythrocyte sedimentation rate of 30 mm at the end of one hour. Ultrasonography of the abdomen confirmed the presence of hepatosplenomegaly and revealed bulky kidneys with prominence of pyramids. The urine examination showed proteinuria (albuminuria 1.4 gm/day), red cell casts and 15-16 red cells and 15 leucocytes per high power field. Serum protein concentration was 4.9 gm/dl with serum albumin concentration of 2 gm/dl and serum cholesterol concentration was 143 mg/dl. Levels of blood urea nitrogen, serum electrolytes, hepatic transaminases and creatinine were normal. The blood level of C₃ was 40 mg/dl (normal >110 mg/dl). Anti-streptolysin O (ASLO) titre was normal. Sickling test and tests for hepatitis B surface antigen, anti-nuclear antibodies and antibodies against double-stranded DNA were negative. Blood culture grew Salmonella typhi and Widal test was positive (Salmonella typhi `O' and `H' titres of 1:240 each). The kidney biopsy revealed diffuse proliferative glomerulonephritis. The patient responded to ceftriaxone and frusemide. The fever disappeared within a week and other clinical abnormalities (hepatosplenomegaly, ascites and anasarca) resolved completely by the 10^th day. At discharge, the patient had no haematuria or pathological albuminuria (urinary protein excretion 63 mg/d). The C₃ level had risen to normal levels.

Discussion

Enteric fever with its classical clinical features hardly ever poses difficulty in the diagnosis. However, when patients present with atypical or rare features, the entity may not be suspected early and institution of treatment may be unduly delayed. Prolonged pyrexia, multi-drug resistance, pneumonia, intestinal perforation and haemorrhage are the commonly encountered problems and complications.

Although neurological complications have been reported, their incidence vary from 5-35% in different series; depending upon the age of the patient and the prevalence of drug resistance.^4,5 Meningismus and acute confusional states are the most frequently encountered neurological complications, which may be present at the time of hospitalisation. Other neurological manifestations that have been reported include seizures, optic neuritis, peripheral neuropathy and focal neurological deficits.⁶ Rarely, cases with facial nerve palsy, palatal palsy and cerebritis have also been reported.^7-9 Most of these neurological complications occur in the second week of fever. A child with typhoid fever presenting with isolated cerebellar ataxia, which although previously noted, is a rarity and account for only 0.05-2% of the cases in certain large series and cases usually occur in the second week.² Para-infectious demyelinating process has been thought to be responsible for this phenomenon.¹⁰ Hyperpyrexia and disturbances of sodium homeostasis are believed to be important contributing factors. The cerebellar manifestations do not require any specific treatment (including steroids) and control of the underlying salmonella infection is all that is required. The majority of patients recover within a few weeks.² What was unusual in our cases was the presence of this complication in the first week of illness which may confuse the treating physician. The complete disappearance of the ataxia with recovery from typhoid fever proved the ataxia to be related to typhoid fever.

Renal involvement with enteric fever is noticed only in 2-3% cases.¹¹ The common complications of typhoid related to the urinary tract include cystitis, pyelitis, pyelonephritis, and mild proteinuria. Glomerulonephritis is uncommon.^3,12 Salmonella typhi has been directly held responsible for glomerulonephritis as renal biopsy studies in these patients have shown diffuse proliferative glomerulonephritis and immunoglobulin, C₃ and Vi antigen deposits in the glomerular basement membrane.^2,3 Most patient with enteric glomerulonephritis present with acute renal failure, hypertensive encephalopathy or nephritic syndrome. In comparison, our case had milder manifestations and showed a prompt recovery.

During the year these cases were encountered, 68 paediatric patients of culture proven enteric fever were treated in our hospital. This suggests the rarity of these complications.

It is important for the treating physician to be informed about these unusual complications of a common disease like typhoid fever, as prompt recognition and early institution of therapy with effective and specific anti-microbial agents results in a complete recovery from complications like cerebellar ataxia and nephritis. While in the cases presented, S. typhi was undoubtedly the cause of cerebellar ataxia and nephritis, these complications are more often encountered with other infections. While the association of nephritis with streptococcal infections is well recognised, infections that cause acute cerebellar ataxia, barring chicken pox, are not well delineated in Indian children. Most often they are associated with viral infections, most likely enteroviral infections, rather than typhoid fever. Cerebellar ataxia can also result from cerebellar space occupying lesions. This fact emphasises that all cases of cerebellar ataxia should be investigated and not treated empirically as typhoid fever.

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