Two Uncommon Paraneoplastic Neurological Syndromes in a Child With Hodgkin Lymphoma

Srinivasan, Arathi; Satish, Gayathri; Scott, Julius Xavier; Madhavan, R. Nirmal; Sathiyasekaran, Malathi

doi:10.1097/mph.0000000000000569

Cited by 6 publications

(5 citation statements)

References 11 publications

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“…4,6 A recent case report from India points at a child with Hodgkin lymphoma who presented with achalasia cardia, and Holmes-Adie pupil occurring synchronously with the cancer. 3 He had pneumatic dilatation treatment twice, had better esophageal function afterwards but no recovery in bilaterally dilated pupils and his visual acuity remained the same despite chemotherapy. Our patient started to swallow efficiently (without radiological recovery) which he couldn't succeed after surgery and has full acuity after chemotherapy.…”

Section: Discussionmentioning

confidence: 92%

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More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma

Demırsoy¹,

Alparslan²,

Şen³

et al. 2019

Turk J Pediatr

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Paraneoplastic syndromes in Hodgkin lymphoma (HL) can be seen with different symptoms and organ findings within a significant time before definite diagnosis of the primary disease. Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported paraneoplastic components in pediatric HL. In this report, we present an 11-year-old girl who had all these three paraneoplastic components synchronously before HL was identified.

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Section: Discussionmentioning

confidence: 92%

“…1 Achalasia, Holmes-Adie pupil, and limbic encephalitis are rarely reported as paraneoplastic components of pediatric HL. 2,3 Hodgkin lymphoma with accompanying limbic encephalitis is named 'Ophelia syndrome'. Herein, we report a child who had achalasia first, developed several neurological symptoms afterwards and finally was diagnosed with HL.…”

mentioning

confidence: 99%

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma

Demırsoy¹,

Alparslan²,

Şen³

et al. 2019

Turk J Pediatr

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“…Limbic encephalitis (LE) and PCD are almost exclusively seen in patients with Hodgkin's lymphoma. Usually, lymphomas are associated with unique paraneoplastic syndromes, but the co-occurrence of paraneoplastic syndromes have also been reported [12,13]. Sensorimotor neuropathies and dermatomyositis are more common in Hodgkin's lymphoma, but the association between Hodgkin's lymphoma and acute motor and sensory axonal neuropathy (AMSAN) has also been recently reported [14].…”

Section: Discussionmentioning

confidence: 99%

A Case of Paraneoplastic Cerebellar Degeneration that Preceded the Diagnosis of Classical Hodgkin’s Lymphoma by 16 Months

et al. 2020

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Objective:Unknown ethiology Background:Paraneoplastic cerebellar degeneration (PCD) is a rare condition that can present as an acute or subacute cerebellar syndrome. PCD is most commonly associated with gynecological and breast cancer, small-cell lung cancer, and classical Hodgkin's lymphoma. The symptoms of PCD can arise several months before tumor diagnosis. This report is of a case of a 44-year-old man with PCD that preceded the diagnosis of classical Hodgkin's lymphoma by 16 months. Case Report:A 44-year-old man was admitted to hospital with a cerebellar syndrome that was initially diagnosed as vertebrobasilar insufficiency. Eight months later, cerebral magnetic resonance imaging (MRI) findings and serum anti-Tr antibodies supported the diagnosis of PCD, but no underlying malignancy was initially found. At 16 months after the initial diagnosis of PCD, the patient developed an enlarged inguinal lymph node. Histology of the excisional lymph node biopsy confirmed the diagnosis of classic mixed cellularity Hodgkin's lymphoma, Ann Arbor stage IIA. The patient responded to four cycles of adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD) chemotherapy. Conclusions:This case illustrates that in patients who present with PCD, an associated malignancy, such as classical Hodgkin's lymphoma, may emerge several months later, which supports long-term follow-up. The presence of anti-Tr antibodies may support a diagnosis of classical Hodgkin's lymphoma in a patient with a history of PCD who develops lymphadenopathy.

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“…PCD is an immune-mediated syndrome, sometimes associated with the anti-Tr antibody. 16 Like other PNS in pediatric HL, the symptoms often resolve with standard therapy. Avramova et al described an eight-year-old boy presenting with dysarthria and diplopia and subsequently diagnosed with HL.…”

Section: Limbic Encephalitismentioning

confidence: 99%

“…16 In this case, they postulated that both manifestations were secondary to immune-mediated damage to the ganglion cells in the myenteric plexus, further proving that PNS can manifest in any number of tissues; in this case, the autonomic system. There was one prior published case of achalasia in a child with HL.…”

Section: Other Neurologic Manifestationsmentioning

confidence: 99%

Paraneoplastic Syndromes in Children with Hodgkin Lymphoma

Betcherman¹,

Punnett²

2017

Oncology &Amp; Hematology Review (US)

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Paraneoplastic syndromes (PNS) refer to a phenomenon whereby certain malignancies manifest as symptoms not directly related to the tumor itself. PNS has been described in association with Hodgkin lymphoma (HL) in adults and children and may affect a number of organ systems. The pathophysiology is variable and in many cases is not well understood. Specific paraneoplastic antibodies have been isolated in some syndromes, though are not required for the diagnosis. The two best described for HL are the anti-Tr and anti-mGluR5 antibodies identified in some cases of limbic encephalitis and cerebellar degeneration respectively. A high index of suspicion for underlying malignancy is necessary when recognizing these clinical syndromes to avoid a diagnostic delay. Successful treatment of the HL often reverses the manifestations of PNS although organ function at diagnosis may limit therapeutic options. Some patients suffer devastating complications of their PNS.

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Two Uncommon Paraneoplastic Neurological Syndromes in a Child With Hodgkin Lymphoma

Cited by 6 publications

References 11 publications

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma

More than Ophelia syndrome: Multiple paraneoplastic syndromes in pediatric Hodgkin lymphoma

A Case of Paraneoplastic Cerebellar Degeneration that Preceded the Diagnosis of Classical Hodgkin’s Lymphoma by 16 Months

Paraneoplastic Syndromes in Children with Hodgkin Lymphoma

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