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Case Report

Indian Pediatr 2011;48: 483-485

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.

 


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.


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
  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
 
Adeno/ ECHO/
CoxsackieB5/Mycoplasma
NCV: nerve conduction velocity; EMG: electromyography.

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

1. Hopkin IJ. A new syndrome: Poliomyelitis-like illness associated with acute asthma in childhood. Aust Paediatr J. 1974;10:273-6.

2. Danta G. Electrophysiological study of amyotrophy associated with acute asthma (asthmatic amyotrophy). J 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

4. Vishwanathan J, Desai AB. Infections and Infectious disease. In: Vishwanathan J, Desai AB. Eds. Achar’s Text book of Pediatrics, 3rd edn. Madras: Orient Longman; 1989.p.351-2.

5. Hensyl WR. Stedman’s Medical Dictionary, 25th edn. Baltimore: Williams and Wilkins; 1990.p.1177.

6. Raghu MB, Balasubramanian S, Menon KG, Gayatri B. Phantom hernia – An unusual manifestation of hypokalemia. Indian Pediatr. 1994; 31: 64-6.

7. Manson J I, Thong Y H. Immunological abnormalities in the syndrome of poliomyelitis-like illness 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 Child. 1980;55:61-74.

9. Acharya AB, Lakhani PK. Hopkins syndrome associated with mycoplasma infection. Pediatr Neurol. 1997;16:54-5.

10. Shahar EM, Hwang PA, Niesen CE, Murphy EG. Poliomyelitis-like paralysis during recovery from acute bronchial asthma: Possible etiology and risk factors. Pediatrics. 1991;88:276-9.
 

 

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