CHRONIC INFLAMMATORY DEMYELINATING POLYNEUROPATHY: A CASE
HIGHLIGHTING IGNORANCE-DRIVEN ETHICAL DILEMMA IN RESOURCE-POOR
SETTINGS
Introduction
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired
immune-mediated disorder of peripheral nerves and nerve roots. CIDP is closely
related to Guillain-Barré syndrome (GBS) and it is considered the chronic counterpart of
that acute disease. CIDP is less common in children than in adults.1 In Japan, the
prevalence rate per 100 000 was 1.61 in the total population; 2.01 in males and 1.23 in
females.1 The age dependent prevalence rate was 0.23 in children less than 15 years old.
Using the American Academy of Neurology (AAN) criteria, the prevalence was 1.97 per
100,000 in this population similar to the prevalence among caucasians.1 Although, the
exact prevalence is not known among African children, there is an apparently similar
immunopathologic basis among different races.1,2 The specific cause of CIPD has not
been identified but there is evidence for its immunologic origin.2 The cellular immunity
is involved through T-cell activation and expression of cytokines while the humoral
immunity via deposition of immunoglobulin and complements on myelinated nerve
fibers.2
The classic form of CIDP manifests a fairly symmetric, predominant motor
involvement of the lower limbs and the deep tendon reflexes are usually reduced or
absent.3 Sensory involvement is common Sensory impairment in CIDP is usually greater
for vibration and position sense than for pain and temperature sense, reflecting the
involvement of larger myelinated fibers.4 Cranial nerve and bulbar involvement occur
in about 10 percent of patients. Constipation and urinary retention are found in late
presentation and severe cases.3,4 Most patients with CIDP exhibit a slowly progressive
course, but a relapsing-remitting course occurs in at least one-third, and more common
in younger patients.4 Electrophysiologic features of CIDP include partial conduction
block, reduction of conduction velocity and compound motor action potential (CMAP)
amplitude while common histologic features are segmental demyelination, onion bulb
formation and axonal degeneration.5 Other relevant investigations include
cerebrospinal fluid (CSF) analysis and MRI of spinal roots, brachial plexus, and
lumbosacral plexus.6
Stringent research criteria of CIDP used in clinical trials often miss the diagnosis
in routine clinical practice leading to late diagnosis and treatment.7,8 Hence, it is
preferable to use clinically oriented tool like the Koski criteria which has specificity and
positive predictive value comparable to both the AAN research criteria and the
European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS)
electrodiagnostic criteria.8 The standard therapies for CIDP are intravenous
immunoglobulins (IVIG), corticosteroids and plasma exchange, but about 15% of cases
are treatment refactory.9, In a large series, prognosis of CIPD varies from complete
remission in 26%, partial recovery in 61% to severe disability in 13%.10 Like other
chronic illnesses, the outcome of CIPD can be influenced by the progression of the
disease itself, , carers’ perception of its aetiology, poverty and sociocultural practices.11
We recently managed a case that shows an intriguing relationship between such
external factors and the outcome of this rare chronic neurological disorder.
Case Report
A nine year old girl (D.T.) was admitted into the paediatric Neurology unit of the
University of Benin Teaching Hospital, Nigeria on the 7th September 2011 with Hospital
Number 546192. She was a bright primary 3 pupil, first of two children of nonconsanguineous parents in social class V.
She first presented in the setting of an upper respiratory tract infection 3 years
earlier when the mother noticed a change in her gait and difficulty with climbing stairs.
Two weeks later, she was admitted due to inability to walk and urinary incontinence.
Neurologic findings were conclusive of a lower motor neuron lesion. The initial
assessment was GBS with a differential diagnosis of transverse myelitis. CSF analysis
showed < 5 white blood cells per mm3, no red blood cells, glucose 52mg/dl and protein
of 49mg/dl. MRI of the spinal cord, electromyography (EMG) and nerve conduction
studies could not be done due to financial constraints. Similarly, plasma exchange and
intravenous immunoglobulin (IVIG) therapies were not achievable. She was closely
monitored and her vital capacity was consistently >20ml/kg. She received physical and
occupational therapies but remained bedridden till the third week on admission when
she was commenced on oral prednisolone 1mg/kg/day, as a suspected chronic variant
of GBS. However, she was discharged against medical advice (DAMA) few days later,
despite social service intervention. She was taken to a traditional healer on a
superstitious basis. Five months into the illness, she recovered partially at home,
walking with a limp till this relapse. She was lost to follow up.
In this second episode, she re-presented with a seven month history of slowly
progressive upper and lower limbs weakness and urinary incontinence; fever and
drowsiness occurred a week before presentation. On examination, she was febrile
(Temperature – 38.50c) with multiple septic decubitus ulcers (grade 2-4, figure 1c).
Neurological examinations revealed altered consciousness (GCS–7/15), hypotonia,
areflexia and zero power in the lower limbs; hyporeflexia and grade 3 power in the
upper limbs. There was loss of pain and vibration sensations up to T4. The diagnosis
was septicemia in a child with CIDP.
Neuroimaging, EMG/Nerve conduction studies, plasma exchanges and IVG’s
therapies were not achieved due to inadequate financial resource. Lumbosacral
radiograph was normal (figure 1a & b) and retroviral screen was negative. She was
managed with intravenous ceftazidine at 150mg/kg/day, gentamin at 5mg/kg/day and
supportive care, but sepsis screen later yielded Kliebsiella species sensitive only to
piperacillin–tazobactam. Fifth day on admission, she had gasping respiration, pulse rate
of 46 beats per minute moderate volume, and percutaneous oxygen saturation of 52%.
Cardiopulmonary resuscitation was commenced but she succumbed to the illness
before transfer to the intensive care unit. Parents declined post-mortem examination.
1a
1b
1c
Figure 1(a): Lumbosacral radiograph AP view of a 9 year old girl with CIDP showing normal
vertebral bodies and pedicles; (b) lateral view showing normal vertebral bodies and spinal
processes; (c) infected stage IV decubitus ulcer in the same patient after DAMA.
Discussion
CIDP is a rare immune-mediated disease in childhood, especially in West Africa
sub-region due to the skew of health care delivery towards control of infectious diseases
while chronic neurologic disorders may be neglected in rural populations. Our patient
manifested typical neurologic features of CIDP following a GBS-like presentation. In a
relapsing course, she had symmetric reduction in deep tendon reflexes, sensory loss and
autonomic dysfunction fulfilling the mandatory clinical criteria of the European
childhood CIPD consortium.12 Also, we confirmed the diagnosis of CIPD in this case
based on her clinical features that fulfilled the Koski criteria which has comparable
validity to both the AAN and EPNS/PNS research criteria.6-8
As found in this patient, cytoalbumin dissociation may occur in up to 90% of
CIDP cases.13 Nerve biopsy for histology, NCV, MRI and genetic studies in selected
cases are useful in excluding differential diagnosis of CIDP.6 This were not done in this
case due to parents limited financial resources and adherence to fictitious beliefs of
“spiritual” or superhuman origin of unremitting illnesses leading to refusal of care.11,
Treatment options in CIDP include corticosteroid which was initiated fourth week into
the illness but was rejected a few days later before any significant response.
Considering common side effects of steroids including impaired wound healing and
immune suppression, specific therapies of sepsis and stabilization were the priorities in
her management on re-admission with extensive decubitus ulcers and lethargy,
following a 3-year long default. Parents could not afford IVIG and plasma exchanges.
She succumbed a few days later apparently due to metastatic complications of
Kliebsiella septicemia and/or progression of CIDP.
Poor prognostic factors in CIDP include a treatment refractory course,
respiratory compromise, CNS involvement and severe co-morbidities.10 Moreover,
resorting to harmful traditional remedies, late presentation and low socioeconomic
status of the carers contributed to poor outcome in this case. Although a legal injunction
can be obtained to admit a child after parents’ presumptuous refusal of treatment
despite adequate counseling, current level of implementation of free health care in our
setting focuses on short-term treatment of acute childhood illnesses precluding chronic
neurological disorders.14,15 Hence, as in this case, the clinician is sometimes in a dilemma
concerning provision of guardianship for an ill child without access to free health
services, if parents declined treatment on erroneous basis.14,15
As illustrated in this case, the outcome of CIDP and other chronic disorders in
childhood in resources-poor settings often hinges on parents’ socio-economic status and
belief systems in spite of clinicians’ advocacy.
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