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Indian Pediatr 2011;48: 483-485 |
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Hopkins Syndrome and Phantom Hernia: A Rare
Association |
KE Elizabeth, CS Guruprasad and TG Sindhu
From Department of Pediatrics, SAT Hospital, Government
Medical College Trivandrum.
Correspondence to: Dr KE Elizabeth, Professor of
Pediatrics, SAT Hospital, Government Medical College,
Trivandrum 695 011, Kerala, India.
Email: [email protected]
Received: November 09, 2009;
Initial review: January 1, 2010;
Accepted: March 29, 2010.
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Acute flaccid paralysis (AFP), other than paralytic poliomyelitis, are
usually due to demyelination like Guillian Barre syndrome (GBS),
transverse myelitis and traumatic neuritis. Poliomyelitis like illness,
Hopkin’s syndrome or Post Asthmatic Amotrophy, associated with bronchial
asthma and hyperIgEemia has been reported in literature. We present a
two and a half year old child who developed AFP with phantom hernia
following an episode of bronchial asthma.
Key words: Acute Flaccid Paralysis, Phantom hernia, Hopkin’s
syndrome, HyperIgEemia.
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S ubsequent to decline in cases
of paralytic poliomyelitis, demyelination is the most common cause
of AFP followed by traumatic neuritis. AFP has also been reported
with asthmatic attack and hyperIgEemia (Hopkin’s syndrome or Post
asthmatic amotrophy) and following ICU admission for critical
illness, called ‘critical illness polyneuropathy/myopathy [1-3].
Phantom hernia refers to unilateral bulging of abdominal wall due to
patchy paralysis of abdominal wall muscles. It was first described
in paralytic poliomyelitis by Achar [4]. It is derived from the word
‘phatasm’ meaning mental imagery produced by fantasy [5]. It has
also been reported with non-polio conditions like hypokalemia
complicating gastroenteritis [6].
We report a case of AFP with phantom hernia
associated with an asthmatic attack and hyper-IgEemia. Several cases
of poliomyelitis like illness following bronchial asthma have
been reported [7-10], but associated phantom hernia has not been
documented.
Case Report
A two-and-a-half year old male child with normal
growth and development was admitted in PICU with acute severe
asthma. He had intermittent asthma for the past one and a half
years. He was fully immunized including pulse polio immunizations.
He had three days of ICU stay and was given nebulization with
salbutamol, ipratropium and steroids, ampicillin, IV methyl
prednisolone and MgSO4
infusion. He responded and did not require any ventilator support or
muscle relaxants.
On the 4th
day of hospital stay, he developed acute flaccid paralysis of the
left lower limb, which progressed and involved the right lower limb
in the next two days. His higher mental function and upper limbs
were normal. He had hypotonia, grade 0 power, areflexia and down
going plantars in both lower limbs. He had no sensory involvement
except for myalgia. Abdominal reflex on the left lower quadrant was
absent. There was transient bladder involvement also. Blood counts
were within normal limits and there was no eosinophilia. CSF study
one week after paralysis showed 0-2 lymphocytes/mm3 and protein of
20 mg/dL and sugar of 80 mg/dL. Mantoux test was negative. Chest
X-ray showed increased bronchovascular markings. Serum CPK and
LDH were normal. Mycoplasma antibody was negative. There was ten
fold rise in serum IgE level, 630 IU/dl (normal <60 IU/dl). MRI
spine and brain were normal. Nerve conduction study showed decreased
amplitude of complex motor neuron action potential with greater
involvement of the left lower limb compared to right. This
asymmetrical pattern and absence of albumin-cytological dissociation
in the second week were inconsistent with GBS. Eventhough
fibrillations were not seen on surface EMG, anterior horn cell
involvement could not be ruled out .The child was non-cooperative
for a needle EMG study.
Para infectious demyelination was considered and
IV Immunoglobulin (IVIG) 2g/kg was given for two days, but there was
no improvement. AFP reporting was done and child was initiated on
physiotherapy. He was discharged on oral steroids. At the time of
discharge, he had severe hypotonia, areflexia and grade 0 power of
both the lower limbs.
During follow up visit 2 weeks later, he was
found to have phantom hernia in the left lumbar region. His stool
culture for poliovius was negative. He is currently on follow up for
the last 6 months with only very little recovery. Phantom hernia has
resolved, but with only Grade 1 power in left lower limb and grade 2
power in right lower limb with muscle atrophy, areflexia, weakness
and severe physical handicap.
Discussion
In 1974, Hopkin described ten cases of
poliomyelitis like illness following acute asthma [1]. Subsequently,
similar cases were reported [2,3,7-10]. All these cases had
hyperIgEemia in common. In these cases, no single organism was
isolated, but microbes like adenovirus, ECHO, Coxsackie B 5 and
mycoplasma were documented. All the reported children had received
various drugs in the recommended limits and no adverse drug
reactions were noted. The unusual combination of symptoms and
striking similarity in these reported cases argue against a chance
association between asthma and paralysis [7,8].
According to a hypothesis put forward by Manson
and Thong, patients who develop poliomyelitis like illness after an
attack of acute asthma, do so because of an underlying immunological
deficiency, albeit of a minor nature [7]. It is possible that due to
further immunosuppression in these patients at the time of stress,
intercurrent infections or corticosteroid therapy, the host
resistance is reduced to such a level that a neurotropic virus, not
usually considered pathogenic, may invade the anterior horn cells
[7].
In view of the persistent clinical course of the
child, with poor response to IVIG and steroids, presence of phantom
hernia, and NCV and CSF findings not consistent with GBS; we thought
of other differential diagnosis of AFP. Paralytic poliomyelitis was
ruled out in this case as the child was fully immunized and stool
culture for poliovirus was negative. Rare association of AFP with
mycoplasma was also ruled out in this case [9]. Due to the
association with bronchial asthma and hyper-IgEemia, Hopkin’s
syndrome was considered. The physical and laboratory findings were
comparable to the previously reported cases of Hopkin’s syndrome,
except for phantom hernia [10] (Table 1).
TABLE I
Previous Cases of Poliomyelitis Like Illness After Acute Asthma
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Reported cases (n-22) |
Gender - M:F |
1.75:1 |
Age at onset |
13 months-11years |
Immunized against poliovirus |
Yes in all cases |
Days between wheezing and paralysis |
4 – 11 |
Meningism |
Yes in 3/22 |
Myalgia |
Yes in 10/22 |
Neurological findings (acute phase) |
Paralysis of arm or leg |
Reduced Motor NCV |
Yes in 3/14 |
Denervation on EMG |
Yes in 13/13 |
Residual weakness with muscle atrophy |
Yes in all cases |
Microbial association
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Adeno/ ECHO/
CoxsackieB5/Mycoplasma |
NCV: nerve conduction velocity; EMG: electromyography.
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Acknowledgment: Dr MK Mohandas, Professor of
Pediatrics, Dr SM CSI Medical College, Karakonam, Trivandrum, Kerala
for his suggestions and, Dr PA Mohammad Kunju and Dr D Kalpana,
Department of Pediatric Neurology, SAT Hospital Trivandrum for their
help in the management of the case.
Contributors: EKE was the consultant
in charge, critically evaluated the manuscript and will act as the
guarantor of study. GCS managed the case, collected data and drafted
the paper. STG helped in management and revision of the manuscript.
Funding: None.
Competing interest: None stated.
References
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illness associated with acute asthma in childhood. Aust Paediatr J.
1974;10:273-6.
2. Danta G. Electrophysiological study of
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Neurol Neurosurg Psychiatry. 1975;52: 728-49.
3. Hermans G, DeJonghe B, Bruyninckx F,
Vandenberghe G. Clinical review: Critical illness polyneuropathy and
myopathy. Critical Care. 2008;12:238-46
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B. Phantom hernia – An unusual manifestation of hypokalemia. Indian
Pediatr. 1994; 31: 64-6.
7. Manson J I, Thong Y H. Immunological
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associated with acute bronchial asthma (Hopkin’s syndrome). Arch Dis
Child. 1980; 55:26-32.
8. Son-Blomquist H K, Bjorksten B.
Poliomyelitis-like illness associated with asthma. Arch Dis
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9. Acharya AB, Lakhani PK. Hopkins syndrome
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10. Shahar EM, Hwang PA, Niesen CE, Murphy EG.
Poliomyelitis-like paralysis during recovery from acute bronchial
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