1
42
sodes of generalised tonic-clonic convulsions, became
deeply unconscious (Glasgow Coma Score of 4), his
pupils became unreactive to light, had global hypertonia
and hyperreflexia with sustained ankle clonus, hyperten-
sion and bradycardia. Due to the suspected raised intrac-
ranial pressure, he was giving hyperventillation and IV
patients with cerebellitis or basal ganglia lesion. Al-
though they (except tremors) were absent in the second
child possibly because of the short duration of stay fol-
lowing convulsion, he had histopathologic features of
cerebellitis.
1
0% mannitol, however, he died about 2 hours after the
Typically, patients with ADEM present with encephalo-
4
,7
onset of convulsion. Lumbar puncture could not be done
before his demise due to the severe cardiopulmonary
instability and suspected intracranial hypertension. Also,
CT scan or MRI was not done because of financial con-
straint.
pathy and cerebellar ataxia. It has been reported that
the neurological presentation in ADEM vary from an
acute explosive onset, with a maximum neurological
deficit attained within 1 day, to more indolent progres-
4
sion with maximum deficit of 31 days. In several re-
ports, most children with ADEM (up to 90 percent) had
polysymptomatic encephalopathy and cerebellar dys-
His autopsy revealed congestion of the terminal
4
,7
1
0 – 15cm of the ileum. The mesenteric lymph nodes
function. They may also present with facial weakness,
dysarthria, dysphagia, ophthalmoplegia, visual impair-
ment arising from optic neuritis; acute flaccid parapar4e,7-
sis and urinary dysfunction resulting from myelitis.
Our first patient had most of these features. Also, cere-
brospinal fluid of this patient showed a mild pleocytosis,
slight protein increase and sterile cultures, which agr4e-6es
with most reports on post-typhoid encephalomyelitis.
were enlarged, but there was no area of ulceration or
haemorrhage. On histology, there was thinning of the
mucosa of the terminal ileum, effacement of its villi and
loss of mucosa glands. The submucosa was infiltrated
by numerous lymphocytes. The mesenteric lymph nodes
as well as the vessels of the submucosa were also con-
gested, in keeping with typhoid ileitis. Grossly, there
was slight oedema of the brain, but no area of haemor-
rhage or necrosis. However, on histology, there was a
widespread demyelination of the brain, especially
around the microglial cells and the small vessels of the
pons, midbrain and cerebellum. The impression was
therefore acute disseminated encephalomyelitis in a
child with typhoid septicaemia.
There are few reports on the brain MRI findings in chil-
dren with ADEM, particularly in the developing nations.
These findings include areas of hyperintense lesions in
the subcortical regions of the brain stem and the cerebel-
4
9
lum. According to Ahmed et al, theyare thought to be
due to cytotoxic and or diffuse vasogenic cerebral oe-
dema. Other MRI findings in typhoid encephalopathy
include bilateral symmetrical hypodensity with slight
hypertrophy of the gyri6 and diffuse cerebral oedema
without any focal lesion.
Discussion
In patients with ADEM, intravenous methylprednisone
or dexamethasone, followed by oral Prednisolone have
been reported to be effective, usually leading to im-
Characteristically, acute disseminated encephalomyelitis
which is a form of post infectious encephalopathy result
from viral infecti5o,6ns. However, the present case reports
4
,10
proved recovery and less disability. The first patient
who had steroid improved steadily with no obvious neu-
rologic sequelae at discharge. Other treatment modalities
include immunomodulatory therapies such as the use of
immunoglobulins and plasmapharesis. The survival rates
of patients with ADEM is usually high, especially if
they are able to overcome the initial acute complications
such a4s,10intracranial hypertension and metabolic derange-
like few others
show that it can also be caused by
bacterial infections such as typhoid fever.
Although, there are many postulations on the pathogene-
sis of CNS involvement in typhoid fever; the exact
4
,
pathogenesis is still unclear. Some of the factors impli-
cated include hyperpyrexia, hypovitaminosis, metabolic
derangements such as hypoglycae,8mia, hyponatremia,
ment.
Sadly, our second patient who deteriorated on
7
hypernatremia and hypocalcemia. Others are toxae-
the 9th day apparently because of these initial complica-
tions, died before the diagnosis of ADEM was made
histopathologically. In undiagnosed and or untreated
patients, the mortality rate may be as high as 20 percent,
and the 7risk of permanent neurological deficits 10 – 30
percent.
mia, cerebral oedema, secondary neuronal changes and
non-specific inflammatory changes in the vessels of the
brain. These non-specific inflammatory changes include
capillary thrombosis, haemorrhage, oedema, and
perivascular infilteration associated with widespread
4
demyelination. Both patients had widespread demyeli-
nation of the brain. This was confirmed in the first pa-
tient by MRI scan of the brain and in the second by
histopathology.
Typhoid fever is associated with several neurological
complications including post-infectious demyelination
as the underlying pathological process. This is usually
reversible but occasionally may be irreversibly lethal if
left untreated. Since clinical diagnosis of ADEM may
not be clear initially and in the absence of brain MRI
scan, paediatricians, particularly those who work in re-
source-poor countries, should consider this diagnosis in
children with typhoid encephalopathy associated with
Typhoid encephalitis has been regarded as a very un-
3
usual complication and it is usually a late feature of
typhoid fever, as shown by our patients.In addition, the
first patient had tremor, choreoathethoid movement,
ataxia and dysdiadocokinesia. These features are seen in