Sandeep Kumar Kar*, Rajat Choudhuri, Dhiman Adhikari,Gargi Nandi and Rajdeep Basu
Department of Anaesthesiology,Pain and Critical Care Medicine, and Department of Cardiac Anaesthesiology Institute of Post Graduate Medical Education and Research, Kolkata, India
Received Date: November 09, 2015; Accepted Date: December 04, 2015; Published Date: December 10, 2015
Citation: Kar SK. Atypical Association of Japanese Encephalitis with Sixth and SeventhCranial Nerve Palsy during Outbreaks : Does it Require a Second Thought? A Case Report. Transl Biomed. 2015, 6:4. doi: 10.21767/2172-0479.100029
Last year in the month of August and September there had been an increased incidence of Japanese Encephalitis being reported all over West Bengal, India. Keeping this public and medical interest in the backdrop, the authors are reporting a 28 years old female patient admitted to the medical ward with acute onset flaccid paralysis of both lower limbs, diplopia, nasal intonation of voice, difficulty in swallowing and features suggesting facial nerve palsy.
The intensivist was called for management of gradually worsening respiratory failure and the patient was put on mechanical ventilator after being shifted to Intensive Therapy Unit. On neurological examination, power of lower limb was diminished with sixth and seventh cranial nerve palsy. The patient was diagnosed to have Japanese Encephalitis infection by a four-fold rise in virus-specific antibody detected in paired acute and convalescent sera by enzyme-linked immunosorbent assay. In nerve conduction velocity test she had bilateral symmetrical demyelinating sensory>>motor polyradiculoneuropathy suggestive of Sural nerve sparing acute inflammatory demyelinating polyneuropathy. Cerebrospinal fluid study revealed albumino-cytological dissociation. She was off the ventilator after 3 days of full course of plasmapheresis and she gradually regained her motor power with physiotherapy and nursing care.
Japanese Encephalitis patients are mainly admitted with feature of central nervous system insults and have poor prognosis. Syndromic association of Japanese Encephalitis with sixth/seventh cranial nerve palsy having Guillain-Barré Syndrome without any features of meningo-encephalitis is a rare phenomenon and there are very few case reports in literature regarding this topic. This case report highlights the successful management of such rare experience.
Japanese encephalitis; Guillain-Barré Syndrome; Sixth and seventh cranial nerve palsy; Plasmapheresis
Japanese Encephalitis (JE) virus is the sole etiologic agent ofJapanese Encephalitis. It is one of the major causes of viralencephalitis in human population . Since the isolation of thisvirus in Japan in 1935, it has spread worldwide becoming amajorpublic health concern . JE is of major public health importancedue to its high epidemic potential, high case fatality rate (CFR),and sequelae among survivors [3,4]. Last year in the month ofAugust and September there has been an increased incidenceof Japanese Encephalitis being reported all over West Bengalwith significant mortality rate. High grade fever with or withoutrigors, headache, general malaise, nausea, vomiting followed byaltered sensorium, convulsions, neck stiffness, muscular rigidity,mask-like facies, and abnormal movements are the classic clinicalpicture of the disease. Apart from the classical presentation other atypical presentations of JE have been reported. Among theman acute flaccid paralysis-like illness with multiple cranial nervepalsy and bulbar palsy has been reported [5-7].
A non-diabetic, non-hypertensive 28 years old female patientwas admitted to the medical ward with features suggestingacute onset flaccid paralysis of both lower limbs, diplopia, nasalintonation of voice, difficulty in swallowing and facial nervepalsy for last 3 days. Weakness started with leg and progressedin ascending order. There was no alteration of sensorium at thetime of admission. Bladder and bowel habits were normal.
There was no history of such neurological disorder running inher family. There was neither history of recent respiratory tractinfection nor gastrointestinal infection. Over the next 72 hours herillness evolved to acute respiratory failure requiring intubationand mechanical ventilation at Intensive Therapy Unit (ITU).
On neurological examination-
Higher function - patient was conscious and orientedMotor functions -power of both lower limbs were 3/5
Power of both upper limbs were 5/5
Sensory functions - bilateral symmetrical distal limb sensory losswere seen which had no definite level.
Reflexes-knee and ankle jerks were diminished, planter reflex wasnon-responsive in both lower limbs,gag reflex was absent.
In cranial nerve examination bilateral 7th (more in left side) and 6th nerve palsy were seen. Bilateral pupillary reflexes were normal.Bilateral palatal movements were sluggish.
There were no abnormality with spine and cranium.
The patient was proved to have Japanese Encephalitis infection bya four-fold rise in virus-specific antibody detected in paired acuteand convalescent sera and CSF by enzyme-linked immunosorbentassay.
Cerebrospinal fluid(CSF) study revealed-protein 66 mg/100ml, glucose 73 mg/100 ml, leukocyte count 5/cu.mm (alllymphocytes).There was no evidence of sepsis in routine bloodinvestigations as suggested by total leukocyte count (TLC) of8400/cu.mm (N69L27M2E2B0). Blood, sputum and urine cultureshowed no growth of organisms. Serial monitoring of serumelectrolytes were unremarkable. Mild hypernatremia was seenwhich was corrected.Haemoglobin value was 12 gm%.
On the 6th day after onset of symptoms, nerve conductionvelocity (NCV) test showed bilateral symmetrical demyelinatingsensory>>motor polyradiculoneuropathy, (upper<<lower)withsuralsensory nerve action potential (SNAP) sparing.
Immediately after admission to ITU one course of plasmapheresiswas done. Simultaneously respiratory therapy, cardiac monitoring,physiotherapy, nutritional supplementation by ryles tube, posturalcare, deep vein thrombosis (DVT) prophylaxis were given. Withtreatment, power and tone of both upper limb and lower limbwere improving. Weaning trials from ventilator were carried onwith continuous monitoring of FEV1, negative Inspiratory force,respiratory rate, vital capacity, tidal volume and she was takenout of ventilator successfully with spontaneous normal breathing pattern and without complication after 3 days of full course ofplasmapheresis. Oral feeding was initiated on the same day. Thepatient was shifted to the ward after gaining normal neurologicalfunction. Patient was discharged and transferred to a local centrefor rehabilitation after 20 days of admission.
Japanese Encephalitis virus is endemic in the Indian subcontinentbut knowledge regarding disease progression still revealsinteresting facts. It is the leading cause of viral encephalitis inAsia, with 30,000-50,000 cases reported annually. Case-fatalityrates range from 0.3% to 60% depending on the age and onpopulation. The natural host of the Japanese Encephalitisvirus is bird and many believe the virus will therefore never becompletely eliminated . Japanese encephalitis is diagnosedby detection of antibodies (IgM) in serum and CSF . There isno specific treatment for Japanese Encephalitis and treatment issupportive, with assistance given for feeding, breathingor seizurecontrol as required.
Japanese Encephalitis typically presents as fever, headache andmental state change due to meningo-encephalitis, but morerarely can also cause a polio-like illness without encephalitis. Wehave reported here a case of a lady who presented with acuteflaccid paralysis due to JEvirus. Electrophysiological studiessuggests that Japanese Encephalitis virus myelitis is caused byanterior horn cell damage and the clinical presentation mimicspoliomyelitis as was our case .
A number of etiological agents such as viral and bacterial infections,vasculitis, surgery, malignancy, vaccination anddrug reactionsare noted to be associated with GBS. Various viral infectionsmay precede the onset of GBS like influenza, cytomegalovirus,Epstein-Barr virus, measles, mumps, adenoviruses etc.
GBS is most commonly characterized by combination of limbparaesthesia, generalized weakness and areflexia. The BrightonCollaboration is an international collaboration sponsored bythe World Health Organization to facilitate the development,evaluation, and dissemination of high quality internationallystandardized case definitions for various illnesses with the aimof improving vaccine safety. These innovatory ‘Brighton criteria’also represents the level of diagnostic certainty based on thepresenting findings at clinical and additional examinations,ranging from level 1 (highest level of diagnostic certainty) tolevel 4 (reported as Guillain-Barré syndrome, possibly due toinsufficient data for further classification) .
Key diagnostic criteria and Brighton casedefinitions for Guillain-Barré syndrome
The findings in our case were consistent with the first, second,fourth, sixth and seventh diagnostic criteria of the BrightonCollaboration as shown in the Table 1.
|Diagnostic criteria||Level of diagnostic certainty|
|1||Bilateral and flaccid weakness of limbs||+||+||+||+/−|
|2||Decreased or absent deep tendon reflexes in weak limbs||+||+||+||+/−|
|3||Monophasic course and time between onset-nadir 12 h to 28 days||+||+||+||+/−|
|4||CSF cell count <50/μl||+||+a||−||+/−|
|5||CSF protein concentration > normal value||+||+/−a||−||+/−|
|6||NCS findings consistent with one of the subtypes of GBS||+||+/−||−||+/−|
|7||Absence of alternative diagnosis for weakness||+||+||+||+|
+ present; − absent; +/− present or absent.
NCS = nerve conduction studies.
a If CSF is not collected or results not available, nerve electrophysiology results must be consistent with the diagnosis Guillain-Barré syndrome.
Table 1: Key diagnostic criteria and Brighton case definitions for Guillain-Barré syndrome
Pathogenesis of GBS not yet fully understood and currentthinking is that GBS may not be a single disease, but a variety ofacute neuropathies with a number of related immune-mediatedpathogenic mechanisms .Most common immunopathologicfindings are endoneural inflammation in spinal nerves roots, distal nerve segments or around potential nerve entrapment sites.Many viral proteins share some cross-reacting determinants withhost tissue components. Asa result, antibodies to self-proteinsare often detected following viral infection. Theoretically, sucha phenomenon would result in autoimmune-mediated tissuedamage. Evidence for the role of molecular mimicry betweensome viral proteins and human myelin basic protein (MBP)has been provided earlier with respect to hepatitis B virus andmeaslesvirus [13,14]. Hence, it is plausible that viruses play amajor role in the pathogenesis of demyelinating diseases. Targetantigens appear to be common to the axon, myelin sheath orboth.The exact antigens, the precipitating event or the resultantmechanism of injury remains somewhat unclear and hencerequires further molecular research.
Virus-specific IgM antibody detection in serum and CSF isrecognised as a sensitive, specific indicator of JEV infection. Theseantibodies can be detected as early as the first day after the onsetof neurological symptoms and over 90% of the JE patients arepositive for this antibody in the CSF by the seventh day of theillness.
About 30% of patients admitted to hospital with Japaneseencephalitis die and around half of the survivors have severe neurological sequelae . About 30% of survivors have frankmotor deficits. 20% of patients have severe cognitive andlanguage impairment (most with motor impairment also) and20% have further convulsions .
Patients affected with Japanese Encephalitis virus are mainlyadmitted with feature of CNS insults and have poor prognosis.This case depicts the rare association of JE with sixth nerve palsyalong with a host of atypical features of Guillain-Barré Syndrome.
On the background of endemic breakouts at various times, thiscase report brings out the notion that atypical presentation ofJapanese Encephalitis might require further research to decipherexact mechanisms of such varied rare presentations.
Keeping this public and medical interest in the backdrop thiscase has highlighted the successful management of such rareexperience.