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CASE REPORT
Year : 2016  |  Volume : 29  |  Issue : 3  |  Page : 762-764

Ptosis and ataxia complicating enteric encephalopathy in a child


Department of Paediatrics, Army College of Medical Sciences, New Delhi, India

Date of Web Publication23-Jan-2017

Correspondence Address:
T Bindu Nair
Department of Paediatrics, Army College of Medical Sciences, Delhi Cantonment, New Delhi - 110 010
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-2098.198805

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  Abstract 

Ptosis, ataxia, and dysarthria presenting together are very rare neurological complications of enteric fever. We report a case of a 3-year-old boy with blood culture-proven enteric septicemia who developed these very rare neurological complications of enteric fever. After starting a course of intravenous antibiotics and steroids, he became afebrile and neurological symptoms abated with no residual effects after 4 weeks. This report is a further addition to all the published neuropsychiatric complications of enteric fever so far.

Keywords: ataxia, enteric fever, neurological manifestations, ptosis


How to cite this article:
Nair T B, Sanjeev K R, Kumar D. Ptosis and ataxia complicating enteric encephalopathy in a child. Menoufia Med J 2016;29:762-4

How to cite this URL:
Nair T B, Sanjeev K R, Kumar D. Ptosis and ataxia complicating enteric encephalopathy in a child. Menoufia Med J [serial online] 2016 [cited 2020 Apr 3];29:762-4. Available from: http://www.mmj.eg.net/text.asp?2016/29/3/762/198805


  Introduction Top


Neuropsychiatric manifestations in enteric fever are the most varied and fascinating of all the complications described. These usually present in the second or third week [1] . Ataxia with ptosis and dysarthria as an isolated early neurological manifestation is very rare. We could find three cases in the literature in the late 1980s and early 1990s; however, none of them had ptosis. We report a case of a 3-year-old boy presenting with ptosis, ataxia, and dysarthria on the third day of a febrile illness. The classical clinical manifestations are rarely seen nowadays, perhaps because of early diagnosis and institution of antibiotic therapy. The epidemiology of the disease is also constantly changing.


  Case history Top


A 3-year-old male child with fever for 3 days developed sudden inability to walk on the third day of his illness. He developed difficulty in swallowing both solids and liquids. Furthermore, he stopped talking or interacting with parents and progressively developed altered sensorium. The boy had no history of headache, vomiting, seizures, decreased vision, or ear discharge. At the time of admission, he was febrile (39.2°C) with a pulse rate of 100/min and was mildly dehydrated. There was no pallor, icterus, or lymphadenopathy on general examination and his anthropometric parameters were within the normal range. The child had mild hepatosplenomegaly. There were no signs of meningeal irritation. Results of the rest of the systemic examinations including fundoscopy were normal.

Investigations on the day of admission (third day of illness) revealed normal hemoglobin level (10.8 g/dl), normal total leukocyte count (6.7 × 10 9 /l), normal differential leukocyte count (polymorphs 68%, lymphocytes 25%, monocytes 3%, and eosinophils 4%), and a lowered platelet count (1 20 000/l). Peripheral smear examination for malarial parasites and rapid malarial antigen tests were negative. Dengue serology was also negative. Serum sodium was 142 mEq/l and K + was 3.5 mEq/l. Urine exam was normal and the urine culture showed no growth. The Widal test was positive (TO and TH titer of 1: 320). Blood culture on day 3 of admission revealed a growth of Salmonella typhi. Cerebrospinal fluid analysis was normal.

The child was treated with ceftriaxone and on day 3 of admission; prednisolone in the dose of 2 mg/kg/day was added with the clinical suspicion of enteric encephalopathy. On day 4 of admission, sensorium improved; he started responding in monosyllables and also started accepting orally. However, he was unable to sit up and developed bilateral ptosis. The pupils and fundi were normal. There was bilateral palatal weakness and speech was dysarthric. Facial and masticatory muscles were not involved. He had a moderate degree of incoordination of all the limbs with gross gait ataxia. Motor power was normal and there was no sensory deficit. There was no nystagmus, hypotonia, or rebound phenomenon.

Ultrasound abdomen showed increased echogenicity of bilateral renal cortex suggestive of medical renal disease and repeat ultrasound after 10 days showed resolution. Electroencephalogram of the child was normal. MRI of the brain revealed widespread large globular lesions affecting the subcortical white matter. The periventricular structures were, however, normal. Moreover, there was no evidence of intraparenchymal or intraventricular collection. There was no contrast enhancement, significant edema, or mass effect. The neostigmine test (0.02 mg/kg neostigmine) was carried out but there was no change in ptosis.

The child progressively improved and on day 10 showed improving ability to hold head, sit, and stand. Drooping of eye lids improved and he started speaking and demanding food and was finally discharged from the hospital on day 14. On the follow-up, the child had normal gait, no drooping of eyelids, or any other neurological deficit. The results of repeat MRI carried out after 2 months was normal.


  Discussion Top


Enteric fever is still a very serious disease in developing countries like India. The mortality in enteric cases still range between 5 and 20% [2] . Global estimates are difficult; however, the greatest estimate of burden of disease is in Asia. The WHO statistics estimate over 22 million cases annually, with at least 200 000 deaths [3] . Annual incidence rates of enteric fever reported from Delhi, India, are up to 980 per 100 000 [4] . It is a common systemic illness with a wide spectrum of presentations and complications. It has a high prevalence in tropical areas such as Asia and Africa. The disease may manifest with complications or in an atypical form like neurological manifestations. This spectrum of the illness constitutes an important but often underdiagnosed component.

The manifestations of enteric fever are often protean and a high index of suspicion is required for diagnosis, especially in endemic areas such as India. Neurological complications in enteric fever are not rare and range from 5 to 35% in various studies [5] . The involvement of the central nervous system in enteric fever has a wide spectrum of presentation. Seizures, delirium, meningism, and coma have been described as common neurological complications of enteric fever but acute ataxia with ptosis and dysarthria as early manifestations of enteric fever is reportedly very rare [6],[7] .

Early encephalopathy is an uncommon and potentially fatal neurological complication. The mechanisms for the neurological manifestations of enteric fever are not well understood. Hyperpyrexia, electrolyte disturbances, neurotoxins, vasculitis, and the autoimmune process are thought to be the mechanisms [8] . A cascade of pathological processes in the cerebrum leads to an acute diffuse encephalomyelitis, causing enteric fever encephalopathy [9] .

The prognosis of neurological deficits in enteric fever is usually good [10],[11] . Most of the neurological complications described were seen during the second or third week of illness, at the height of fever, or during defervescence. Some occurred during convalescence like neuropathy, amnesia, and psychosis. Others like motor neuron disease and scholastic deterioration occurred well after recovery [12],[13] . In our patient, the neurological deficit occurred early during the first week of the illness. Perhaps the clinical spectrum and epidemiology is changing because of casual and unscientific use of antibiotics for every febrile illness by local practitioners even without confirming diagnosis.

Appropriate antibiotic treatment is important to cure enteric fever with minimal complications. The choice of drug and the duration of therapy depend on several factors such as the clinical severity of the case, the patient's condition, and drug resistance. The increasing antibiotic resistance of S. typhi is a concern. The treatment of enteric fever with third-generation cephalosporins, such as ceftriaxone, is associated with higher cure rates and lesser incidence of morbidity and mortality. No specific therapy other than supportive therapy is recommended for neurological manifestations. The prognosis of neurological deficits in enteric fever is usually good. In most of the cases the recovery is slow but complete ([Figure 1] and [Figure 2]).
Figure 1: Child with enteric fever showing ptosis.

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Figure 2: Child 2 months later, after recovering from ptosis.

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Lakhotia M, Gehlot RS, Jain P, Sharma S, Bhargava A. Neurological manifestations of enteric fever. J Indian Acad Clin Med 2003; 4 :196-199.  Back to cited text no. 1
    
2.
Parry CM, Hien TT, Dougan G, White NJ, Farrer J. Typhoid fever. N Engl J Med 2002; 347 :1770-1782.  Back to cited text no. 2
    
3.
Crump JA, Luby SP, Mintz ED. The global burden of typhoid fever. Bull World Health Organ 2004; 82 :346-353.  Back to cited text no. 3
    
4.
Sinha A, Sazawal S, Kumar R, Sood S, Reddaiah VP, Singh B. Typhoid fever in children aged less than 5 years. Lancet 1999; 354 :734-737.  Back to cited text no. 4
    
5.
Haque A Neurological manifestations of enteric fever. In: Chopra JS, Sawhney IMS, editors. Neurology in the tropics. 1 st ed. New Delhi: BI Churchill Livingstone; 1999:506-512.  Back to cited text no. 5
    
6.
Wadia RS, Ichaporia NR, Kiwalkar RS, Amin RB, Sardesai HV. Cerebellar ataxia in enteric fever. J Neurol Neurosurg Psychiatry 1985; 48 :695-697.  Back to cited text no. 6
    
7.
Sawhney IMS, Prabhakar S, Dhand UK, Chopra JS. Acute cerebellar ataxia in enteric fever. Trans R Soc Trop Med Hyg 1986; 80 :85-86.  Back to cited text no. 7
    
8.
Osuntokun B, Bademosi O, Ogunremi K, Wright S. Neurosychiatric manifestation of typhoid fever in 959 patients. Arch Neurol 1972; 27 :9-13.  Back to cited text no. 8
    
9.
Bansal AS, Venkatesh S, Jones SR, Williams W. Acute aphasia complicating enteric fever in an adult. J Trop Med Hyg 1995; 98 :392-394.  Back to cited text no. 9
    
10.
Ali G, Rashid S, Kamli M, Shah P, Allaqaband Q. Spectrum of neuropsychiatric complications in 791 cases of typhoid fever. Trop Med Int Health. 1997; 2 :314-318.  Back to cited text no. 10
    
11.
Hauser SL, Asbury AK. Guillain-Barré syndrome and other immune-mediated neuropathies. Harrison's principles of internal medicine 19 th ed. New York, NY: McGraw Hill; 2015. 2694.  Back to cited text no. 11
    
12.
Haque A. Neurological manifestations of Enteric fever. In: Chopra JS, Sawhney IMS, editors. Neurology in tropics. 1 st ed. New Delhi: BI Churchill Livingstone; 1999. 506-512.  Back to cited text no. 12
    
13.
Worku B. Typhoid fever in an Ethiopian Children's Hospital, 1984-1995. Ethiop J Health Dev 2000; 14 :311-313.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2]



 

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