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Case presentation and literature review

Dr Huma Mir
PGR peads department
GMMMC Sukkhur

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 History

5 years old boy Abdul Rayan,weight 15 kg , resident of panoaqil admitted

through emergency with the complains of ;

High grade fever

Generalized tonic clonic fits. 7 days

Altered level of consciousness

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 History of presenting illness
• For history informant is a mother of child who did not have any formal
education.
• My patient was in usual state of health 7 days ago when he developed
fever high grade ,sudden in onset ,continues not associated with chills or
rigors ,along with generalized tonic clonic fit’s, frequency of fits was 4 to 5
episodes per day , duration of fits ranges from 30seconds to 2 mins , fits
were associated with frothing , no history of urinary or fecal incontinence .
. he was having numbness of both the lower limbs and upper limbs
followed by weakness of both limbs and inability to walk .
• According to mother child was admitted at panoaqil (private
hospital)where fits frequency were controlled after administration of some
injectables ,also Lumber puncture (kamer sy pani nikal k test Kiya tha)test
was performed,which was normal.

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 History of presenting illness

Patient developed altered level of consciousness during stay at hospital

associated with stiffness of whole body , there is no previous history of any
such episode , he remained admitted there for 6 to 7 days and referred to
civil hospital sukkhur for further evaluation and management. During course
of admission at panoaqil hospital he was treated with iv antibiotics and anti
epileptics ..

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 Past Medical History

History of fever ,cough and respiratory distress for

that admitted in private hospital for 15 days ago.
Ño previous history of similar complains.
No other history of any illness, hospital admission or
drug history.

Past surgical history not significant.

 :Birth history
Born via LSCS at full term no history of birth asphyxia or NICU admission
after birth. Breastfed for 2 years.

Developmental History :
No history of delayed milestones , developmental milestones were
achieved at appropriate age.

Vaccination history :
As per EPI upto date . BCG scar mark present.

Family history,
4th product of consagnious marriage.
No history of similar complains infamily.No other significant family history.
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 General Physical Examination
On GPE , ill looking child of average built disoriented in time place and
space ( not obeying commands) , tonic posturing , GCS(E2V2M2)6/15 lying
in bed with cannulation done on dorsum of left hand ,having no dysmorphic
features has following vitals :

HR : 98 bpm Anthropometry
Temp : 99.2 F. Weight= 15 kg
BP : 100/70 mmhg
SpO2 : 95 % Height=95 cm
RR : 18 / min ( No distress )
BSR : 101 mg / dl
CRT : < 3 sec

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 General Physical Examination
Head to Toe Examination
No dysmorphic Features
No signs of dehydration , jaundice , pallor , cyanosis or distress
No purpuric spots , rash
No radio radial or radio femoral delay
No central or peripheral cyanosis , clubbing
BCG scar mark present
No lymph nodes palpable
Good oral hygiene
Central Trachea ,no deviation
No muscle wasting ( upper and lower limbs )

GAIT : Cannot walk

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 CNS Examination
Inspection : No visible deformity , nerurocutaneous leisions , involuntary movements ,
muscle wasting or hyperytrophy , muscle bulk normal with no fasciculations.

Palpation : No muscle temderness

Tone : Increased tone of both upper and lower limbs

Power : 1/5 of both lower limbs and 2/5 of both upper limbs

Reflexes : Plantars upgoing , deep tendon reflexes were brisk and exaggerated , clonus
was not illicitable. Abdominal reflexes were intact and bladder was not palpable.

Cranial nerves exam was unremarkable and signs of meningeal irritations were absent

Rest of the Systemic Examination was Normal

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 CASE SUMMARY

5 years old boy ,Vaccinated, developmentally up todate with the

complains of High grade fever, Generalized tonic clonic fits and Altered
level of consciousness for7 days
Disoriented in time ,place and person GCS 6/15
CNS examination,
Increased tone in both upper and lower limbs
Planters upgoing, Deep tendon reflexes were brisk and
exaggerated .
Power 1/5 in lower limbs and 2/5 in upper limbs
Rest of vitals along with systemic examination intact.
RRe

Differential diagnosis
 Differential Diagnosis

1;Partially treated meningitis

,Bacterial / Viral / Tuberculous meningitis

2. Autoimmune encephalomyelitis

3.ADEM (Acute disseminated encephalomyelitis)

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 Laboratory Investigations

CBC with peripheral smear,

Hb ;9mg /DL
MCV; 66
MCH; 20
TLC;17000
Lymphocytes;80%
Platelet count; 400000
 CSF RE

Cell count :; 6
Predominantly lymphocytes
Glucose; 56mg/DL
Proteins; 86mg/dl

CSF C/S ; Negative culture,No growth yielded

 Investigations

CRP : 27
LFT : Normal
RFT : Normal

1. CEMRI BRAIN = Multifocal abnormal signal intensity lesions ( white

matter hyperintensity lesions) were noted in bilateral frontal – cortical
region , bilateral basal ganglia , post limb of internal capsule , left
thalamus and body of corpus callosum .

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Investigations

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 Provisional Diagnosis

ADEM (Acute Disseminated

Encephalomyelitis)

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 Treatment
Admitted in ward , managed symptomatically.

1 . Empirical antibiotic and antiviral therapy ( Inj Meronem , Inj Vancomycin

in meningitic doses , inj Aklova )

2 . Antiepileptics ( Inj lerace , Inj phenytoin ),fits were controlled Then

shifted to oral form of antiepileptic

3 . Inj Methylprednisolone (30 mgkg) for 5

days ,then on tapering dose of steroids , injectable stopped
4 . Caloric intake record .
5. Supplements added in the treatment,(syp lederplex ,syp osobion)
6. Bladder and bowel care
.
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Demyelinating Disorders of Central Nervous system
Annual incidence of 0.5-1.66 per 100,000 children.
Neurologic dysfunction caused by immune mediated attacks on
the white matter insulating the brain , optic nerves and spinal
cord.
cont:
There are two igG antibodies recognized as
playing an important role in demyelination,
aquaporin 4-antibody (AQP4-Ab) and myelin
oligodrendrocyte glycoprotien antibody (MOG-
Ab)
 The majority of children presenting with an episode of
demylination are monophsic & they do not relapse.
Monophasic demyelinating disorders of childhood
include
acute disseminated encephalomyelitis (ADEM),
optic neuritis(ON),and
transverse myelitis(TM)
Relapsing forms of demyelination include
MS and
neuromyelitis optica spectrum disorders(NMOSD)
Acute Disseminated
Encephalomyelitis (ADEM)
ADEM is an inflammatory, demylinating event of early
childhood presenting with an acute onset of polyfocal
neurologic deficit, accompanied by encephalopathy and
changes compatible with demyelination on brain MRI.
Is an autoimmune disorder in which the body’s
immune system mistakenly attacks its own
brain tissue, triggered by an environmental
stimulus in genetically susceptible individuals
Epidemiology
 Mean age 5 and 8 yr
 Annual incidence range from 0.1-0.6 per 100,000
per year in paediatric population.
 ADEM is usually monophasic, but recurrence
can occur.
 If the recurrence is 3 months or longer after 1 st
episode, the condition is termed multiphasic
disseminated encephalomyelitis(MDEM)
 Epidemiology
 50% of ADEM are associated with MOG-Ab
positive in serum.
 Almost all cases of MDEM are MOG-Ab
positive.
 An episode of ADEM can also be followed by
non-ADEM demyelination in a new loaction.
› MOG-Ab negative =MS
› Optic Nerve = ADEM-ON
› ON & spinal cord= NMOSD
 Pathogenesis

 Molecular mimicry induced by infectious

exposure or vaccine has been thought to trigger
production of cns autoantigens although casuality
has never been proven.
 Many pts experience a transient febrile illness in
the month prior to ADEM onset.
 PATHOGENESIS

 Molecular mimicry induced by infectious

exposure or vaccine has been thought to trigger
production of cns autoantigens although casuality
has never been proven.
 Many pts experience a transient febrile illness in
the month prior to ADEM onset.
 post infectious ADEM
 In approximately 50-70 percent of ADEM
cases,the inflammatory attack is preceded by a
viral or bacterial infection.
 A seasonal distribution has been observed
showing that most ADEM cases occur in winter
and spring
 The inflammatory attacks and neurological
symptoms often begin with a couple of weeks
after viral or bacterial infections.
 post infectious ADEM

Large no of viruses associated with these infections,

including
Measles , Mumps, Rubella
Varicella zoster, Epstein-Barr, Cytomegalo virus ,
Herpes simplex, Hepatitis A , Influenza ,
Enterovirus infections.
 post Immunization ADEM
 Associated with immunization for..
 Rabies , Hepatitis B , Influenza
 Diptheria / pertussis / Tetanus
 Measles , Mumps, Rubella
 Pneumococcus ,Polio , smallpox and varicella.
 Catogaries Of ADEM
 Monophasic ADEM Is a one time episode that
can develop over a period for as long as three
months
 Any new or changing symptoms within three
months period considered as one event
 Symptoms that might occur an oral steroid taper
or within one month of the completion of the
taper are also classified as one single episode.
 Recurrent ADEM
 Is defined as a subsequent attack that involves the
same symptoms that occurred during the initial
attack
 The MRI findings tend to be similar to the initial
attack and there are no lesions, but there could
be an enlargement of the lesions from the original
episode.
 Multiphasic ADEM
 Is defined as an attack that involves new areas of
the central nervous system from the initial or
previous attacks
 There must be signs of encephalopathy

,but symptoms and neuroimaging findings are in

different areas from the initial attack.
There might be new lesions evident on MRI
 Clinical manifestations
 Lethargy
 Fever
 Headache
 Vomiting
 Meningeal signs
 Seizures including status epilepticus
 Encaphalopathy is the hallmark of ADEM
 Focal neurological deficits include
 Irritibility , confusion , Altered level of consciousness visual
loss, cranial neuropathies, ataxia,motor and sensory deficits ,
bowel/bladder dysfunction with concurrent spinal cord
demylination
Other Common neurological signs of ADEM

 Long tract pyramidal Signs

 Acute hemiparesis
 Cerebellar ataxia
 Cranial neuropathies optic neuritis
 Spinal cord dysfunction transverse myelitis.
Less common neurological signs of ADEM

 Aphasia
 Movement disorders
 Sensory deficit
 Less common neurologic signs of ADEM include

 Aphasia
 Movement disorders
 Sensory deficit
 Neuroimaging
 Head CT scanning may be normal or show
hypodence regions.
 Cranial MRI, exhibits bilateral, large multifocal
and sometimes confluent , edematous mass-like
T2 lesions with vareable enhancment with white
and gray matter of the cerebral hemispheres,
cerebellum and brainstem.
 The spinal cord may have an abnormal T2 signal
or enhancement with or without clinical signs of
myelitis.
neuroimaging
 Laboratory investigations

 Non specific findings of inflammation

 Leukocytosis is common , occuring in up to two
thirds of patients
 Prdominantly lymphocytosis
 C reactive protein may be increased
 ESR is elevated in a one third of patients
 Laboratory Investigations
 CSF findings are often normal or can exhibit
pleocytosis with lymphocytic and monocytic
predominance.
 Elevated immunoglobulin production can be present,
but true Oligoclonal bands (OCBs )positivity is rare
most often associated with Multiple sclerosis.
 EEG show generalized slowing, consistant with
encephalopathy, although polyregional
demylination of ADEM can also cause focal slowing
or epileptiform discharges.
 Differential Diagnosis
 CSF findings are often normal or can exhibit
pleocytosis with lymphocytic and monocytic
predominance.
 Elevated immunoglobulin production can be present,
but true Oligoclonal bands (OCBs )positivity is rare
most often associated with Multiple sclerosis.
 EEG show generalized slowing, consistant with
encephalopathy, although polyregional
demylination of ADEM can also cause focal slowing
or epileptiform discharges.
 Laboratory findings
 CSF findings are often normal or can exhibit
pleocytosis with lymphocytic and monocytic
predominance.
 Elevated immunoglobulin production can be present,
but true Oligoclonal bands (OCBs )positivity is rare
most often associated with Multiple sclerosis.
 EEG show generalized slowing, consistant with
encephalopathy, although polyregional
demylination of ADEM can also cause focal slowing
or epileptiform discharges.
 Teatment
 High dose i/v steroid are commonly employed
typically methylprednisolone 20-30 mg/kg per
day for 5 days with a max dose of 1000mg per
day followed by an oral prednisolone taper of 1-2
mg/kg/day(max 40-60 mg/day) over 4-6 wks.
 i/v immunoglobulins (usually 2g/kg over 2-5
dyas) or plasmapheresis(typically5-7 exchanges
every other day) for refractory and severe cases
 It is important to first considered a
treatment with antibiotics and or
acyclovir untill a infectious cause is
ruled out.
 Plasma Exchange (PLEX) ,is
recommended if there is no response
to corticosteroids
 It is important to first considered a
treatment with antibiotics and or
acyclovir untill a infectious cause is
ruled out.
 Plasma Exchange (PLEX) ,is
recommended if there is no response
to corticosteroids
 Extended Follow up
 Follow up Magnetic resonance imaging (MRI)
shows complete or partial resolution of
abnormalities in the majority of ADEM
cases.some expert suggest obtaining at least two
additional MRIs after the initial MRI ,over a
period of atleast Five years from the initial
episode of ADEM.
 Prognosis

 Most children experience full motor recovery

after ADEM , but residual defects can be seen
and cognitive and behaverial changes are not
uncommon. Recovery starts within days to weeks
, but symptoms can fluctuate.
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