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 Table of Contents  
DR. WATSONS’S CLINICAL MYSTERY
Year : 2021  |  Volume : 1  |  Issue : 3  |  Page : 209-211

Pattern recognition of bizarre eye movements: Greatly used but meagerly utilized


1 Department of Pediatrics, Armed Forces Medical College, Pune, Maharashtra, India
2 Department of Pediatrics, Command Hospital (Southern Command), Pune, Maharashtra, India

Date of Submission21-Jul-2021
Date of Decision30-Jul-2021
Date of Acceptance11-Aug-2021
Date of Web Publication31-Aug-2021

Correspondence Address:
Dr. Vishal Sondhi
Department of Pediatrics, Armed Forces Medical College, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ipcares.ipcares_232_21

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How to cite this article:
Kurup A, Badal S, Sondhi V. Pattern recognition of bizarre eye movements: Greatly used but meagerly utilized. Indian Pediatr Case Rep 2021;1:209-11

How to cite this URL:
Kurup A, Badal S, Sondhi V. Pattern recognition of bizarre eye movements: Greatly used but meagerly utilized. Indian Pediatr Case Rep [serial online] 2021 [cited 2021 Sep 26];1:209-11. Available from: http://www.ipcares.org/text.asp?2021/1/3/209/325090


  Scene of the Crime Top


An 11-month-old, developmentally normal girl presented with a history of loss of attained milestones over 10 days. Premorbidly, the infant had started walking independently, had attained pincer grasp, was uttering bi-syllables, and playing lap games like “peek-a-boo.” The illness had started with progressively increasing unsteadiness of gait, increased falls, and then loss of ability to walk unassisted, over 48 h. Concurrently, she lost the ability to reach for and successfully pick up her toys and stopped uttering bi-syllables. Her parents noticed that she had become very irritable, her sleep was interrupted, and she had started “startling” easily. There was no history of fever, loss of consciousness, seizures, projectile vomiting, visual or hearing impairment, facial asymmetry, or paucity of movements of any limb. There was a significant history of a febrile illness with low grade fever, cough, and coryza for a few days (sans any rash), 2 weeks before the onset of these symptoms. History of trauma, rash, or accidental ingestion of any drug was not found. There was no history of similar complaints in the past or in any close contacts. The child was the firstborn of a nonconsanguineous marriage. There was no family history of unexplained early childhood deaths, developmental delay or regression, visual/hearing impairment, or intellectual disability.

Dr. Watson: It seems like we are dealing with neuroregression. I wonder what lies in store for us in the clinical examination.

Examination revealed an extremely irritable infant with normal vitals (absence of fever and hypertension) and age appropriate for anthropometry. The anterior fontanelle was open, of normal size and at level. Pallor was seen. There was no jaundice, dysmorphic facies, neurocutaneous markers, or rash. On neurological examination, the sensorium was normal. Chaotic eye movements lasting for 1–2 s were observed [Video 1][Additional file 1]. There were no cranial nerve deficits. The fundus was normal. Motor evaluation revealed decreased truncal and lower limb tone, power of >3/5 in the lower limbs, and preserved deep-tendon jerks. There was no apparent sensory impairment to touch or pain. Multiple cerebellar signs were noted; titubation, dysmetria, intention tremors, and truncal ataxia. She did not exhibit any signs of raised intracranial pressure or meningeal involvement. There were no palpable masses or organomegaly. The remaining systemic examination was within the normal limits.

Dr. Watson: By Jove! We are dealing with ataxia of 10 days duration! Let me review the causes of acute/sub-acute ataxia. It may even be the first episode of recurrent ataxia.

The leading causes of acute/recurrent ataxia in children include brainstem malignancies or encephalitis, postinfectious/immune phenomena, and a few select genetic disorders [Box 1]. In addition, trauma and vascular events such as cerebellar hemorrhage should be ruled out.



Dr. Watson: Hmm…I couldn't find anything! What do I need to see in “those eyes?”

The three aberrant eye movements include opsoclonus, ocular flutter, and nystagmus. Opsoclonus (also known as “dancing eyes” or saccadomania) is a form of ocular dyskinesia. It is described as sudden, repetitive, involuntary, high-amplitude, chaotic, arrhythmic, and multi-directional (i.e., upward, downward, and torsional) conjugate saccadic ocular movements. This results in oscillopsia (objects appear to jiggle or vibrate when in actuality they are still) and visual blurring. Opsoclonus is present during fixation (while looking at a stationary object), pursuit movements (while looking at a moving object), and convergence (while looking at an object closely). It persists during sleep or closure of the eyelids.[1] To differentiate opsoclonus from nystagmus and ocular flutter, one must appreciate the number of planes of involvement and the character of the movements. Ocular flutters are saccadic and the oscillations exist in only the horizontal plane, in contrast to opsoclonus which are multi-planar. Nystagmus, on the other hand, is a rhythmic, rapid jerk followed by a slow corrective saccade.

Dr. Watson: Very well, what are other things associated with these dancing eyes.

Opsoclonus should alert the clinician to elicit history of and look for myoclonus (of the limbs and trunk), behavioral changes (usually irritability), and sleep disturbances. One should look fortremor and hypotonia, both of which were present in this case.[2] The detection of a palpable lump on abdominal examination and hypertension is indicative of a neuroblastoma.

Dr. Watson: All right then, let us synthesize all that we have learned till now.

We have a premorbidly healthy 11-month-old girl with acute-onset regression of motor and speech milestones, excessive irritability, altered sleep pattern, easy “startling,” intermittent opsoclonus, lower limb and truncal hypotonia, truncal ataxia, and cerebellar signs. Eureka! The clinical phenotype matches that of opsoclonus myoclonus ataxia (OMA). Other differentials will need to be excluded (toxic-metabolic encephalopathies, structural diseases [metastasis, inflammation, demyelination, and hemorrhage] within the pons/cerebellum).

Dr. Watson: At last, we are making some progress! Let us plan investigations.

The hematological profile and acute-phase reactants were normal. Thus, we did not feel the need to perform a lumbar puncture. The liver and kidney function tests were normal. Normal magnetic resonance imaging (MRI) of the brain excluded cerebellar lesions. The screening protocol for the detection of a neuroblastoma was followed: The 24-h urinary vanillylmandelic acid and homovanillic acid were elevated; MRI (neck, chest, abdomen, and pelvis) identified a lobulated retroperitoneal mass (2.1 cm × 2.8 cm × 3.9 cm) that was abutting the inferior vena cava and encasing the right renal vein [Figure 1]. This suggested a neuroblastoma of adrenal origin, which was confirmed by increased uptake in a 123-Iodine-meta-iodobenzylguanidine scan. A computed tomography-guided biopsy of the lesion showed the typical histopathology of a ganglioneuroblastoma. The anti-Hu antibody levels were positive on the qualitative analysis. The final diagnosis was a paraneoplastic OMA secondary to adrenal ganglioneuroblastoma.
Figure 1: The image depicts a T2-weighted magnetic resonance image of the abdomen in the axial (a) and coronal planes (b and c). The images demonstrate a lesion (white arrowhead) abutting the IVC (gray arrows) and encasing the right renal vein, suggestive of right adrenal origin neuroblastoma

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Dr. Watson: Great. Now let us manage her according to standard protocol.

A multi-specialty team was involved in the management. For the OMA, she was administered a single dose of intravenous immunoglobulin (IVIG) and rituximab. Adrenocorticotropic hormone (ACTH) was started. The symptoms resolved with 1 week of initiation of therapy. Specific management of the gangioneuroblastoma included neo-adjuvant chemotherapy for 2 months, surgery (total resection of the tumor and adrenalectomy with preservation of the adrenals on the unaffected side) and chemotherapy for 6 months. She was continued on ACTH for 12 months. Two years have elapsed, and till date, there has not been any recurrence of irritability, and so far, she is developing typically.


  Discussion Top


OMA is an exceedingly rare condition (0.27–0.40/million children/year) that mainly affects young children (mean age 1.5–2 years). Although multiple underlying paraneoplastic, autoimmune, and infectious etiologies[3] are described, majority of cases are idiopathic.[4] The disease is characterized by irregular multidirectional eye movements without intersaccadic intervals, associated with myoclonus, cerebellar ataxia, and behavioral abnormalities. Early detection and management are crucial in view of its neuropsychiatric and neurological effects, as well as the risk of a possible hidden malignancy. Almost 50% children with OMA have an underlying neuroblastoma, and 2% of such children develop paraneoplastic OMA.[5] Other malignancies associated with OMA include ganglioneuroblastoma and ganglioneuroma. Postinfectious OMA is known to follow infections with Streptococcus, Epstein–Barr virus, Mycoplasma pneumoniae, Hepatitis C, Adenovirus C3, Rotavirus, and others. CNS autoimmunity has been demonstrated in OMA, with pathological expansion of B-cells and oligoclonal bands in the cerebrospinal fluid of children with the disease.[6]

The management of OMA encompasses multiple immunotherapeutic agents, including corticosteroids, IVIG, azathioprine, cyclophosphamide, and occasionally rituximab (supposedly decreases relapses).[7] Keeping in mind, the long-term neurological sequelae (residual psychomotor retardation and behavioral abnormalities in 60%–80%), long-term immunosuppression may be mandated in most cases of OMA. In children with unclear etiology and an initial negative paraneoplastic workup, tumor surveillance should be aggressive during the follow-up to avoid relapses.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Amin OS, Shwani SS. Opsoclonus. BMJ Case Rep 2017;2017:bcr2017219433.  Back to cited text no. 1
    
2.
Pike M. Opsoclonus-myoclonus syndrome. Handb Clin Neurol 2013;112:1209-11.  Back to cited text no. 2
    
3.
Hero B, Schleiermacher G. Update on pediatric opsoclonus myoclonus syndrome. Neuropediatrics 2013;44:324-9.  Back to cited text no. 3
    
4.
Krug P, Schleiermacher G, Michon J, et al. Opsoclonus-myoclonus in children associated or not with neuroblastoma. Eur J Paediatr Neurol 2010;14:400-9.  Back to cited text no. 4
    
5.
Rudnick E, Khakoo Y, Antunes NL, et al. Opsoclonus-myoclonus-ataxia syndrome in neuroblastoma: Clinical outcome and antineuronal antibodies-a report from the children's cancer group study. Med Pediatr Oncol. 2001;36:612-22.  Back to cited text no. 5
    
6.
Wells EM, Dalmau J. Paraneoplastic neurologic disorders in children. Curr Neurol Neurosci Rep 2011;11:187-94.  Back to cited text no. 6
    
7.
Pranzatelli MR, Tate ED. Dexamethasone, intravenous immunoglobulin, and rituximab combination immunotherapy for pediatric Opsoclonus-Myoclonus syndrome. Pediatr Neurol. 2017;73:48-56.  Back to cited text no. 7
    


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