case rePOrt ACUTE DISSEMINATED ENCEPHALOMYELITIS- MYRIADS OF - - PDF document

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case rePOrt

ACUTE DISSEMINATED ENCEPHALOMYELITIS- MYRIADS OF PRESENTATION Mukul Aggarwal, Vikrant Sood, K C Aggarwal, Archana Aggarwal* Abstract This retrospective case series of 6 patients illustrates various clinical presentations of acute disseminated encephalomyelitis (ADEM). The patients presented with acute onset of focal neurological defjcit (3 patients), psychosis (1 patient) and ataxia (1 patient) and visual loss (1 patient). Magnetic Resonance Imaging (MRI)

• f brain showed characteristic radiological changes.

Complete recovery was seen in 5 patients, with no evidence of recurrence on follow up. Considering such a myriad presentations, this entity should always be considered in differential diagnosis of acute

• encephalopathy. MRI should form a part of workup in

all cases. Key words: Acute disseminated encephalomyelitis, methyl prednisolone, MRI. Introduction Acute disseminated encephalomyelitis (ADEM) is an acute immune mediated demyelinating disease with a variable clinical presentation. It is a self-remitting disease that follows viral infection or rarely vaccination. No clinical or laboratory feature is pathognomonic and computed tomography (CT) scan of head is not sensitive for diagnosis. MRI brain should be considered early in patients with acute onset of unexplained encephalopathy or focal neurological defjcit. It has improved prognosis with immunomodulatory agents like steroids. Patients in our series, diagnosed as ADEM, had variable unusual polysymptomatic presentation and their clinico-radiological profjle is presented here, which has been sparingly reported from this part of country. Case Series A retrospective analysis of consecutive patients presenting with acute onset fever, altered sensorium with variable neurological defjcit, at a tertiary care centre in Northern India, was done over a period of 6 months from January 2009 to June 2009. Detailed examination with investigations [fundus examination, cerebrospinal fmuid (CSF) analysis, CT and MRI head] were done. Once a diagnosis of ADEM was made using Krupp Criteria (1), patients were put on methyl prednisolone (25 mg/kg) with supportive management. Patients were followed up monthly over 1 year and follow up MRI was done at 3 and 12 months, post diagnosis. Six cases (age varying between 4-11 years) were diagnosed as ADEM as per the criteria. Three patients presented with upper motor neuron type quadriparesis with bowel or bladder involvement, 1 with abnormal behavior and unsteady gait each and one with acute

• nset of visual loss (due optic neuritis with relative

afferent papillary defect positive in both eyes and visually evoked potentials showing bilateral delayed p-100 wave). Sensory loss was detected in one

• patient. One patient had right upper motor neuron

type facial palsy (case 2). (See table 1). Work up for tuberculosis and malaria was negative in all cases. CSF examination was normal in 4/6 patients, 1 had raised proteins (case 4) and 1 showed CSF pleocytosis (case 6). CT head was abnormal only in 1 patient only (case Case No. Clinical Findings MRI Picture 1. Fever, weakness, bladder/ bowel disturbance for 15 days Patchy ill-defjned intramedullary hyperdensities from D2 to D9 level. hyperintense foci on T2 scattered in periventricular sub cortical and deep white matter 2. Fever, weakness, bladder/ bowel disturbance for 5 days Multiple T2 hyperintense foci in bilateral supratentorial, sub cortical and deep white matter, bilateral cerebral peduncles, posterior pons, right superior cerebellar and left middle cerebellar peduncles 3. Abnormal behavior, fever for 7 days T2 hyperintense foci scattered in bilateral periventricular sub cortical and deep white matter in frontal and parietal lobes 4. F e v e r, h e a d a c h e , unsteadiness for 15 days T2W hyperintense foci in bilateral cerebellar white matter, brainstem, thalami and basal ganglia 5. Fever, weakness, bladder/ bowel disturbance for 4 days Two hypointense foci on T1 in Right Corona radiata. On T2, intense central signal. Spinal cord in cervicothoracic region shows thickening +T2 hyperintensity with patchy enhancement. 6. Fever, sudden visual loss for 7 days Multiple focal areas of altered signal intensities (hyperintense) on T2/ FLAIR images in bilateral subcortical white matter, basal ganglia and right thalamus. Table 1: Clinical History and MRI Findings

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1 had bilateral white matter edema) while MRI brain showed changes of ADEM in all (see table 1). Two patients had spinal cord involvement (case 1, 5). Five patients had complete recovery both neurologically and developmentally, while one with severe neurological defjcit had partial recovery only (case 2). Discussion ADEM is a monophasic, polysymptomatic immune- mediated demyelinating disorder. (2) The mean age at presentation is 6-8 years. (2-4) Typical cases of ADEM arise 1-20 days after a febrile infectious illness (commonly following respiratory or gastrointestinal) presumed to be of viral etiology, including rubeola, rubella, varicella, herpes etc. (2,5) It may follow immunizations which include rabies, pertussis, measles, Japanese B virus, tetanus, and infmuenza. (5) Hepatitis B vaccine as a possible cause has been recently refuted. (6) Only one-third of our patients had preceding illness of upper respiratory tract or diarrhea while none received vaccine in recent past. The fjrst signs of ADEM usually include abrupt onset

• f irritability and lethargy. It has varied presentations,

as evident by our study, ranging from involvement of cerebrum (seizures, psychiatric features, hemiparesis) and brainstem (cranial nerve involvement) to spinal cord (paraparesis). (2-4) Presentation with altered behavior (in form of psychosis), as in one of our cases, is extremely rare. It may have typical cerebellar/ meningeal signs and optic neuritis (though only 1/6 had

• ptic neuritis in our study). (7) Relapsing/ multiphasic

ADEM (MDEM) has been described in some studies. (2) ADEM is now being diagnosed more frequently with the broader use of MRI which typically shows T2 enhancing disseminated multifocal lesions in the white matter, basal ganglia, thalamus, and brainstem consistent with edema, infmammation, and demyelination. (2,4,6) Sometimes initial MRI may be normal and then later characteristic lesions appear. Thus, appearance

• f new lesions during recovery may not represent
• recrudescence. CSF fjndings are non-diagnostic, with

majority having mild pleocytosis and normal to raised

• proteins. (5) Only two of our patients had abnormal CSF
• fjndings. The CT scan shows low-density abnormalities

in white matter, but frequently may be normal as in

• ur series. The differential diagnosis should include

encephalitis, multiple sclerosis (MS), progressive multifocal leukoencephalopathy, cerebrovascular accident, acute infmammatory demyelinating poly- radiculoneuropathy and cranial thrombotic lesions. The biggest challenge is to differentiate with the 1st episode

• f MS. Features favoring diagnosis of MS include later

age at presentation (second decade), focal signs without signifjcant encephalopathy, periventricular and corpus callosum lesions on MRI, appearance of new MRI lesions after resolution of initial episode. The IgG (intrathecal) index, IgG synthetic rate, or oligoclonal bands are positive in majority of fjrst MS episodes and in >90% cases with recurrent attacks. (2,7,8) These studies are positive in <30% of ADEM cases. (2,3,7) CSF cytokines analysis is an upcoming modality for this differentiation. (9) The outlook for recovery is generally excellent. More than 50% of children recover without disability within 6 months (2,7), the remainder may have residual

• disability. Mortality is <5% (10,11). Prognosis for

complete recovery is poor in children younger than 2 years, polysymptomatic presentation, progressive evolution, patients with myelitis, and those who have signifjcant edema of the brain or spinal cord. (2) Most of our patients (5/6) had complete recovery. Supportive care forms the cornerstone of therapy with immunomodulatory pharmacotherapy rapidly being recognized as an important adjuvant. These may include high-dose intravenous corticosteroids (methylprednisolone) and intravenous immunoglobulin (IVIG). Alternative approaches include (1) combination

• f intravenous corticosteroids and IVIG, (2) cyclosporin,

(3) cyclophosphamide, or (4) plasma exchange/

• plasmapheresis. (10,12,13)

Contributors: KCA conceived the idea and critically evaluated the manuscript. MA and VS collected the data and prepared the manuscript. AA helped with the radiological diagnosis and also evaluated the

• manuscript. MA will act as guarantor of the study. The

fjnal manuscript was approved by all authors. Funding: None Confmicting Interests: None stated References

• 1. Krupp LB, Banwell B, Tenembaum S; International

Pediatric MS Study Group. Consensus defjnitions proposed for pediatric multiple sclerosis and related disorders. Neurology 2007; 68: 7-12

• 2. Dale RC, Branson JA. Acute disseminated encephalomyelitis
• r multiple sclerosis: can the initial presentation help in

establishing a correct diagnosis? Arch Dis Child 2005; 90:636-639

• 3. Tenembaum S, Chamoles N, Fejerman N. Acute

disseminated encephalomyelitis: a long-term follow-up study of 84 pediatric patients. Neurology 2002; 59: 1224- 1231

• 4. Singhi PD, Ray M, Singhi S, Khandelwal NK. Acute

Disseminated Encephalomyelitis in North Indian Children: Clinical Profjle and Follow-Up. J Child Neurol 2006; 21: 851-857 5. Hartung HP, Grossman RI. ADEM: distinct disease or part

• f the MS spectrum? Neurology 2001; 56: 1257-1260
• 6. Mikaeloff Y, Caridade G, Suissa S, Tardieu M. Hepatitis B

vaccine and the risk of CNS infmammatory demyelination in childhood. Neurology 2009; 72:873-880.

• 7. Dale RC, Sousa DC, Chong WK, Cox TCS, Harding B, Neville
• BGR. Acute disseminated encephalomyelitis, multiphasic

disseminated encephalomyelitis and multiple sclerosis in

• children. Brain 2000; 123: 2407-2422
• 8. Jan MMS. Childhood multiple sclerosis. J Pediatr Neurology

2005; 3: 131-136

• 9. Leake JAD, Albani S, Kao AS, Senac MO, Billman GF et
• al. Acute Disseminated Encephalomyelitis in Childhood:

Epidemiologic, Clinical and Laboratory Features. Pediatr Infect Dis J 2004; 23: 756-764

• 10. Jones CT. Childhood autoimmune neurologic diseases of

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the central nervous system. Neurol Clin. 2003; 21: 745- 764

• 11. Maramattom BV, Sarada C. Clinical features and outcome
• f acute disseminated encephalomyelitis (ADEM): An
• utlook from South India. Ann Indian Acad Neurol 2006;

9: 20-24

• 12. Lin CH, Jeng JS, Yip PK. Plasmapheresis in acute

disseminated encephalomyelitis. J Clin Apher. 2004; 19: 154-159

• 13. Finsterer

J, Grass R, Stolberger C, Mamoli B. Immunoglobulins in acute parainfectious disseminated encephalomyelitis. Clin Neuropharmacol 1998; 21: 258-261

From: Department of Pediatrics and *Department of Radio-diagnosis, VMMC and Safdarjang Hospital, New Delhi, India. Address for Correspondence: Dr Mukul Aggarwal, 107, Bank Enclave, New Delhi- 110092, India. Email: mukulmamc@gmail.com. E-published: 1st February 2012. Art#7 DOI No. 10.7199/ped.oncall.2012.7

case rePOrt

ICTAL SYNCOPE Ahmed Riaz Abstract Isolated syncope is a rare manifestation of seizures in children. Many times, such episodes are mistaken as primary cardiac dysarrhthymias and we report one such case in an infant, who succumbed to the illness, despite treatment. Key words: Ictal syncope, Electro encephalography (EEG), Electrocardiography (ECG), Sick Sinus Syndrome (SSS) Introduction Episodic loss of consciousness presents as a diagnostic challenge to the physicians as well as to the neurologists, many a time. Often, such episodes are named “epileptic” and even treated wrongly, depending upon the specifjc and non-specifjc inter-ictal EEG changes. When symptoms keep recurring, despite treatment, one would consider other organic and non-

• rganic causes and invariably subject the patients to

many diagnostic procedures with very little positive

• results. Changes in cardiac rhythms are known to occur

during clinical seizures, but in situations where there is an epileptic discharge in EEG along with bradycardia and syncope, it is often referred as ictal bradycardia syndrome or ictal syncope. (1) Seizure related cardiac arrhythmias are mostly encountered in older children and adults only and very rare in infants. (2) The given case in an infant describes the initial diffjculty in arriving at a diagnosis and the subsequent outcome. Case Report A six months old girl was referred from a primary physician for recurrent breath holding attacks since the age of 4 months. She was born as term, IUGR and at 4 months age, she started developing frequent apnea, following prolonged incessant cry with momentary stiffness and up rolling of eyes. These episodes were diagnosed as “breath holding spells” even though there were no defjnite precipitating events. Routine ECG and EEG could not reveal initially any specifjc abnormality except bradycardia; she received oral iron syrup for some time with no improvement of symptoms and so parents stopped the drug. At six months of age, symptoms became more severe and of prolonged durations; at one instance she was hospitalized when she developed prolonged apnea, needed oxygen inhalation and bag and mask ventilation. At that time, ECG revealed, prolonged sinus bradycardia, asystole, junctional escapes preceded by sinus tachycardia. Sick sinus syndrome (SSS) was contemplated on this basis and oral beta-blocker (propranolol) treatment was started. Later permanent cardiac pacemaker was implanted (Intermedics S.A., Mode VV1 DASH rate adaptive pulse generator with rate of 80/mt) following left thoracotomy with an intramyocardial unipolar

• lead. With this, her symptoms improved and so she

was labeled as SSS. After 2 weeks, she had a severe life threatening event; she collapsed with bradycardia and lost consciousness suddenly. She was intubated and ventilated for 48 hours. Pacemaker function was checked and found to be normal. Phenobarbitone was given as loading dose with the probable diagnosis

• f autonomic seizures as, interestingly, a prolonged

sleep EEG recording with video telemetry, at this time, revealed a left fronto temporal paroxysmal theta burst which was not documented before; this confjrmed a diagnosis of ictal syncope. Carbamazepine was planned; however the next day, she developed one more prolonged episode of syncope, bradycardia and died as she could not be resuscitated. Discussion Syncope as an isolated manifestation of seizures in children has a poor localizing value and the term ictal syncope is proposed for transient loss of consciousness and posture that occurs in a seizure before or even without convulsions. (1) Seizure related cardiac dysrhythms are well recognized and present mostly as tachyarrhythmia and bradycardia; asystole, on the