Emergency. 2018; 6 (1): e23 CA S E RE P O RT Hopkins Syndrome in a 14 Year Old Boy; a Case Report Alireza Majidi1, Sasan Saket2, Zohreh Nabizadeh Gharghozar3, Ehsan Akrami2, Seyed Mohsen Fayyazi1∗ 1. Emergency Department, Shohadaye Tajrish Hospital, Shahaid Beheshti University of Medical Sciences, Tehran, Iran. 2. Pediatric Neurology Research Center, Shahaid Beheshti University of Medical Sciences, Tehran, Iran. 3. School of Nursing and Midwifery, Kashan University of Medical Sciences, Kashan, Iran. Received: August 2017; Accepted: January 2018; Published online: 16 April 2018 Abstract: Hopkins syndrome (HS) is a flaccid paralysis resembling poliomyelitis that has been seen in some children who are recovering from an acute episode of asthma. This syndrome should be suspected based on clinical findings even before the occurrence of characteristic breathing patterns and epilepsy. We report a 14-year-old boy who had experienced an episode of HS. Keywords: Pitt-Hopkins syndrome; nervous system diseases; rare diseases; asthma © Copyright (2018) Shahid Beheshti University of Medical Sciences Cite this article as: Majidi A, Saket S, Nabizadeh Gharghozar Z, Akrami E, Fayyazi SM. Hopkins Syndrome in a 14 Year Old Boy; a Case Report. Emergency. 2018; 6 (1): e23. 1. Introduction In some children recovering from an acute episode of asthma, a flaccid paralysis resembling poliomyelitis has been encountered (1-3). This condition, first reported from Aus- tralia in 1974, has been termed Hopkins syndrome (HS) (4). No consistent virus has been cultured from such patients, who have generally been successfully vaccinated against po- liomyelitis (5). The disorder primarily affects the anterior horn cells. A rapid progression of paralysis usually affects one limb, leaving the child with a severe and permanent weak- ness. Sensation is preserved; the cerebrospinal fluid (CSF) usually shows moderate mononuclear pleocytosis, and the protein content can be slightly elevated (6-8). Magnetic reso- nance imaging (MRI) changes in the anterior horn have been documented (9). Some of the children have shown evidence of an underlying immune deficiency (10). We report a 14- year-old boy who had experienced an episode of HS. 2. Case Presentation: A 14-year-old boy with a history of asthma since being 2 years old was admitted to hospital with history of seizure and loss of consciousness. There was a history of common cold in the ∗Corresponding Author: Seyed Mohsen Fayyazi; Emergency Department, Shohadaye Tajrish Hospital, Shahrdari Avenue, Tajrish Square, Tehran, Iran. Tel: 00989306224313 Email: mfayyazib@yahoo.com patient a week before and he had experienced severe short- ness of breath 4 days before admission. No significant his- tory of trauma was recorded. His mother had a history of abortion. His mother had no certain disease during and be- fore being pregnant with him. He had used atrovent and salbutamol sprays. No family history of similar illness was present. At the time of admission, vital signs were as fol- lows: temperature 37.8 ◦c, pulse 131 beats per minute, respi- ratory rate 26 per minute and blood pressure 90/50 mmHg. On physical examination, he was confused and there was bilateral expiratory wheezes as well as scattered rhonchi in the lower lobes. His thoracic x-ray was normal. The neuro- logic examination results were normal. His complete blood count was as follows: 25,600 white cells/mm3, with 81% neu- trophils, 2% eosinophils, 15% lymphocytes, and 3.0% mono- cytes, Erythrocyte sedimentation rate (ESR) was (8, 0-10 mg/dl), C-reactive protein (CRP) was (4.1 mg/dl, 0-6 mg/dl), pH= 7.59, Po2= 96.5, HCO3= 25.9 mmol/l, and O2sat=94%. Other routine laboratory test results were normal. The an- tiviral antibodies in paired serum samples showed no sig- nificant changes in any of the examined viruses, including herpes 1 and 2, echovirus, enterovirus, coxsackievirus, and poliovirus types 1, 2, and 3. TORCH, anti-mycoplasma, and anti-borrelia titers were normal. Hbs Ag, Abs Ab, HCV Ab and HIV Ab were negative. The cerebrospinal fluid cultures were negative. No cardiac abnormalities were noted on clinical and ultrasound exam- inations. The sleeping EEG showed anomalies in the fron- tocentral regions with left prevalence and tendency toward This open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0). Downloaded from: www.jemerg.com A. Majidi et al. 2 contralateral diffusion. Abdominal ultrasound and com- puted tomography (CT) scans were normal. His brain MRI was normal. With suspition to Pitt-Hopkins Syndrome Acy- clovir 400 mg was prescribed. He experienced no other asthma and seizure attack. During a 5-month follow-up from the episode there were no new clinical symptoms. 3. Discussion Hopkins syndrome is a rare disease affecting anterior horn cells following an acute asthma attack, most often in children that have atopic asthma (11). Generally, this disease mani- fests from several days to a few weeks after an acute attack of asthma, presenting as the acute onset of flaccid paralysis, and in most cases progresses to severe muscle atrophy in the affected limb (12). Based on previous studies, in such episodes, there is a poor response to corticosteroids and usually there are no recur- rences (13). Some cases have predominant ventral root in- volvement on MRI, while other cases exhibit an extension of the lesions into the anterior columns (14-16). Therefore, the site of lesions in HS is not strictly limited to the ante- rior horn. In some of the previous studies it was reported that peripheral blood lymphocytes of patients with asthma express more cytokine on stimulation with allergens com- pared to those with skin allergy (17), but currently there is no evidence-based clinical correlation between cytokine pro- duction and Hopkins syndrome. Atopic myelitis (AM) is a clinical entity with similarity to Hopkins syndrome. Many reports have confirmed the presence of evidence in the cen- tral nervous system or peripheral nervous system in patients with a high level of total IgE and co-existing atopic disease (18). HS and AM differ from each other in the preferential age of onset, neurologic manifestations, and preferential sites of spinal cord involvement. However, both conditions are sim- ilar regarding the most important point that myelitis devel- ops in the presence of atopic disorders, which suggests a link between atopy and the development of spinal cord inflam- mation. Both of them could be an allergic mechanism due to cross reactivity between an allergen and CNS or PNS. Kira et al. described 22 patients with myelitis of unknown etiology and atopic diathesis, 5 of whom showed focal amyotrophy in one or two limbs (16). Hopkins syndrome is frequently associated with epilepsy. Previous reports do not include detailed EEG descriptions, but slow-wave activity was reported in the patients of Pitt and Hopkins (1978), whereas in Singh’s (1993) patient, there was “generalized epileptogenic activity”. Horiuchi et al. described a 22-year-old woman who showed an additional episode of myelitis after another asthma attack, although no relapse has ever been reported in HS cases (4, 19, 20). Joubert Syndrome, Rett Syndrome and Angelman Syn- drome should be considered as differential diagnoses in pa- tients with the same symptoms as our patient (21). 3.1. Conclusion: Hopkins syndrome should be suspected based on clinical findings even before the occurrence of typical breathing pat- terns and epilepsy. 4. Appendix 4.1. Acknowledgements All the staff members of the emergency department of Shohaday-e-Tajrish Hospital are thanked for their coopera- tion throughout the study period. 4.2. Authors contribution All authors meet the standard criteria of authorship based on the recommendations of the international committee of medical journal editors. 4.3. Conflict of interest The authors declare that there is no conflict of interest in any phase of performing the study. 4.4. Funding None. References 1. Hopkins I. A NEW SYNDROME: POLIOMYELITISâĂŘ- LIKE ILLNESS ASSOCIATED WITH ACUTE ASTHMA IN CHILDHOOD. Journal of Paediatrics and Child Health. 1974;10(5):273-6. 2. Yeung SC, Antonio G, Ip KS. Flaccid paralysis of the limbs after an asthmatic attack. Pediatric neurology. 2010;42(2):133-6. 3. 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This open-access article distributed under the terms of the Creative Commons Attribution NonCommercial 3.0 License (CC BY-NC 3.0). Downloaded from: www.jemerg.com Introduction Case Presentation: Discussion Appendix References