The common etiology associated with acute facial nerve paralysis in children are otitis media, mastoiditis, viral infections such as herpes, varicella, mumps, HIV, meningitis, encephalitis, mycoplasma, Lyme disease, and inflammatory conditions such as vasculitis, Henoch–Schonlein purpura, Kawasaki syndrome, Gullain–Barre syndrome, and hypertension.[3]
Seventy percent of the children with acute lower motor neuron facial paralysis have a favorable prognosis and it resolves spontaneously within 3 months without any sequela. In 40%–75% of children, cause of unilateral facial paralysis is idiopathic, described as Bell's palsy and most of them have a positive history of viral illness 2–3 weeks preceding the neurological manifestations. The use of steroids to reduce the duration of paralysis and reduce the risk of long-term impairment was based on adult studies though the benefits of steroids in children is yet to be proven.[4] Due to the self-resolving nature of the idiopathic variety of the facial palsy seen in children, the neurological manifestations of leukemia as an etiology is never thought of. Steroids, when commonly prescribed in such cases causes a partial recovery thereby masking the primary pathology and adds to the diagnostic dilemma.
The frequency of symptomatic facial palsy has been found to be higher in the younger age group when compared to the idiopathic variety.[5]
AML accounts for 15% of all leukemia and presents with symptoms of prolonged fever, hepatosplenomegaly, and skin or mucocutaneous bleeds. When focal masses of immature myeloid cells from the granulocytic lineage infiltrate the soft tissues and bones, they are called granulocytic sarcomas or chloromas and occur in 5% of cases of AML. It is postulated that the granulocytic sarcomas traverse through the haversian canals from the bone marrow and gets deposited in the subperiosteum to form soft tissue masses.[6]
Granulocytic sarcomas may manifest concurrently with the disease or during remission or relapse. However, when sarcomas precede the disease in peripheral blood or marrow, it often poses a diagnostic challenge, more so, if cranial neuropathies are caused by unidentifiable chloromas as in our case. Facial paralysis resulting from leukemic infiltration, though rare, occurs during the relapse of the disease or as a complication primary disease, but it is not a well-recognized presenting symptom of childhood leukemia. Diagnostic delays of 1 month have been reported when facial nerve palsy was the isolated manifestation of AML in children.[7] Otomastoiditis due to the leukemic infiltration of the temporal bone has been attributed to the facial nerve paralysis. MRI with contrast of the facial nerve canal helps in identifying the facial nerve enhancement. However, the clinical findings of facial nerve paralysis were not always associated with radiological findings in most of the cases.[7]
Baek et al. have reported 11 children who had facial nerve paralysis as isolated feature of AML. Brain imaging studies showed mastoiditis in four of them and chloroma was identified in five of them. Six of them did not have blast in the CSF. Facial nerve palsy improved within a mean period of 1–6 months of chemotherapy.[7] Rohit et al. have reported a case of 13-year-old girl-AML with t(8:21) positivity who presented with bilateral proptosis and facial nerve palsy.[8]
When leukemic children presented with cranial neuropathies, the treatment included systemic and intrathecal chemotherapy with whole brain irradiation. However, Baek et al. recommend allogenic bone marrow transplant, avoiding whole brain irradiation in children to reduce the development of secondary malignancies and to prevent the long-term sequelae on cognitive and endocrine function.
Due to the self-resolving nature of the idiopathic variety of facial nerve palsy, when these children present to general physicians or neurologists, the diagnosis of leukemia is overlooked. Gradual progression of the paralysis beyond 3 weeks should warrant additional investigations to rule out the etiology.
| CASE REPORT | |
|
Bell's palsy in an adolescent girl - not always a neurologist's territory: A case report and review of literature
Latha M Sneha1, Raichel Priyanka2, Shanthini Thanga Tamilselvan2, Julius Xavier Scott3
1 Department of Pediatrics, Division of Pediatric Hemato Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India
2 Department of Pediatrics, Sri Ramachandra University, Chennai, Tamil Nadu, India
3 Division of Pediatric Hemato Oncology, Sri Ramachandra University, Chennai, Tamil Nadu, India
| Date of Web Publication | 13-Jul-2017 |
     |
Correspondence Address:
Latha M Sneha
Division of Pediatric Hemato Oncology, Sri Ramachandra University, No. 1, Ramachandra Nagar, Porur, Chennai - 600 116, Tamil Nadu
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/cjhr.cjhr_123_16
| Abstract |
Infections, inflammatory, and autoimmune conditions are the well-recognized etiologies of acute facial nerve paralysis in children. Bell's palsy is idiopathic peripheral facial nerve palsy. Cranial neuropathies do occur in children due to the central nervous system involvement by malignancies but uncommon in pediatric acute lymphoblastic leukemias and even rarer in acute myeloid leukemias (AMLs). We report a case of a 13-year-old girl who presented with acute facial nerve palsy, was being treated as Bell's palsy elsewhere and was later diagnosed to have AML.
Keywords: Acute myeloid leukemia, Bell's palsy, child
| How to cite this article: Sneha LM, Priyanka R, Tamilselvan ST, Scott JX. Bell's palsy in an adolescent girl - not always a neurologist's territory: A case report and review of literature. CHRISMED J Health Res 2017;4:209-11 |
| How to cite this URL: Sneha LM, Priyanka R, Tamilselvan ST, Scott JX. Bell's palsy in an adolescent girl - not always a neurologist's territory: A case report and review of literature. CHRISMED J Health Res [serial online] 2017 [cited 2017 Jul 14];4:209-11. Available from: http://www.cjhr.org/text.asp?2017/4/3/209/210478 |
| Introduction |  |
Acute, peripheral facial palsy can be presenting feature of infections, inflammatory, and autoimmune conditions and has a good prognosis in children. The incidence of facial paralysis in children <10 years of age is reported to be 2.7/100,000.[1] Majority of them are unilateral, idiopathic, and termed Bell's palsy. A diagnosis of exclusion, Bell's palsy accounts for 42%–85% of cases in children with facial nerve paralysis.[2] Although association of facial palsies in malignancies is well reported, facial paralysis is not a well-recognized presenting feature of leukemias in children, especially in acute myeloid leukemia (AML). The presence of Bell's palsy in children warrants a complete evaluation to rule out leptomeningeal diseases. We report a case of an adolescent girl who presented with acute facial nerve palsy, treated as Bell's palsy elsewhere and was later diagnosed to have AML.
| Case Report | |
A 13-year-old girl presented with acute onset of right-sided facial nerve palsy of 2 weeks duration. She was diagnosed to have idiopathic Bell's palsy elsewhere and was being treated symptomatically with physiotherapy and oral steroids with no improvement in symptoms. She had no constitutional symptoms of fever, anorexia or fatigue, bone pain, mucocutaneous, or skin bleeds. In view of persistent symptoms, she was referred to a higher center for further evaluation. Examination revealed a right-sided lower motor neuron facial nerve palsy [Figure 1], without hepatosplenomegaly or lymphadenopathy. Central nervous system examination and rest of the systemic examination were normal. Investigations revealed hemoglobin 5.9 g/dl, total leukocyte count 14,900/μL (polymorphs: 64.1%, lymphocytes 30.3%), and platelet count 83,000/μL. Peripheral smear revealed leukocytosis with increase in blasts (50%) with Auer rods More Details along with thrombocytopenia [Figure 2].
 | Figure 1: Right-sided lower motor neuron palsy Click here to view |
 | Figure 2: Peripheral smear showing blasts with Auer rods Click here to view |
Bone marrow aspiration showed hypercellular marrow with 58% blasts, promyelocytes - nil, myelocytes - 9, metamyelocytes - 9, neutrophil - 3, eosinophil - 2, band - 4, lymphocytes - 9, monocytes - nil, and plasma cells - 1 [Figure 3]. Cerebrospinal fluid (CSF) analysis was negative for malignant cells. Flow cytometry revealed blasts positive for CD33, cytoplasmic myeloperoxidase, and Human leukocyte antigen –D related (HLA-DR). Magnetic resonance imaging (MRI) brain, MRI angiogram, and venogram were normal. Cytogenetics was negative for t(8:21), inversion 16, t(9:11), and t(15:17). The girl was diagnosed as AML M0. She was started on chemotherapy and after the first cycle of chemotherapy, the bone marrow is in remission and she had partial resolution of facial nerve palsy.
 | Figure 3: Bone marrow aspirate showing hypercellular marrow with increased blasts 58% Click here to view |
| Discussion | |
The common etiology associated with acute facial nerve paralysis in children are otitis media, mastoiditis, viral infections such as herpes, varicella, mumps, HIV, meningitis, encephalitis, mycoplasma, Lyme disease, and inflammatory conditions such as vasculitis, Henoch–Schonlein purpura, Kawasaki syndrome, Gullain–Barre syndrome, and hypertension.[3]
Seventy percent of the children with acute lower motor neuron facial paralysis have a favorable prognosis and it resolves spontaneously within 3 months without any sequela. In 40%–75% of children, cause of unilateral facial paralysis is idiopathic, described as Bell's palsy and most of them have a positive history of viral illness 2–3 weeks preceding the neurological manifestations. The use of steroids to reduce the duration of paralysis and reduce the risk of long-term impairment was based on adult studies though the benefits of steroids in children is yet to be proven.[4] Due to the self-resolving nature of the idiopathic variety of the facial palsy seen in children, the neurological manifestations of leukemia as an etiology is never thought of. Steroids, when commonly prescribed in such cases causes a partial recovery thereby masking the primary pathology and adds to the diagnostic dilemma.
The frequency of symptomatic facial palsy has been found to be higher in the younger age group when compared to the idiopathic variety.[5]
AML accounts for 15% of all leukemia and presents with symptoms of prolonged fever, hepatosplenomegaly, and skin or mucocutaneous bleeds. When focal masses of immature myeloid cells from the granulocytic lineage infiltrate the soft tissues and bones, they are called granulocytic sarcomas or chloromas and occur in 5% of cases of AML. It is postulated that the granulocytic sarcomas traverse through the haversian canals from the bone marrow and gets deposited in the subperiosteum to form soft tissue masses.[6]
Granulocytic sarcomas may manifest concurrently with the disease or during remission or relapse. However, when sarcomas precede the disease in peripheral blood or marrow, it often poses a diagnostic challenge, more so, if cranial neuropathies are caused by unidentifiable chloromas as in our case. Facial paralysis resulting from leukemic infiltration, though rare, occurs during the relapse of the disease or as a complication primary disease, but it is not a well-recognized presenting symptom of childhood leukemia. Diagnostic delays of 1 month have been reported when facial nerve palsy was the isolated manifestation of AML in children.[7] Otomastoiditis due to the leukemic infiltration of the temporal bone has been attributed to the facial nerve paralysis. MRI with contrast of the facial nerve canal helps in identifying the facial nerve enhancement. However, the clinical findings of facial nerve paralysis were not always associated with radiological findings in most of the cases.[7]
Baek et al. have reported 11 children who had facial nerve paralysis as isolated feature of AML. Brain imaging studies showed mastoiditis in four of them and chloroma was identified in five of them. Six of them did not have blast in the CSF. Facial nerve palsy improved within a mean period of 1–6 months of chemotherapy.[7] Rohit et al. have reported a case of 13-year-old girl-AML with t(8:21) positivity who presented with bilateral proptosis and facial nerve palsy.[8]
When leukemic children presented with cranial neuropathies, the treatment included systemic and intrathecal chemotherapy with whole brain irradiation. However, Baek et al. recommend allogenic bone marrow transplant, avoiding whole brain irradiation in children to reduce the development of secondary malignancies and to prevent the long-term sequelae on cognitive and endocrine function.
Due to the self-resolving nature of the idiopathic variety of facial nerve palsy, when these children present to general physicians or neurologists, the diagnosis of leukemia is overlooked. Gradual progression of the paralysis beyond 3 weeks should warrant additional investigations to rule out the etiology.
| Conclusion | |
While managing young children with acute lower motor neuron facial nerve palsy, neurologists and general physicians should have an index of suspicion for the neurological manifestations of acute leukemia and hence complete blood counts, peripheral smear and a bone marrow study if needed should be a part of the work up for etiology in such cases. The routine use of steroids may result in partial remission and can cause diagnostic delays.
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 | |
| 1. | |
| 2. | May M, Fria TJ, Blumenthal F, Curtin H. Facial paralysis in children: Differential diagnosis. Otolaryngol Head Neck Surg 1981;89:841-8.  |
| 3. | Ciorba A, Corazzi V, Conz V, Bianchini C, Aimoni C. Facial nerve paralysis in children. World J Clin Cases 2015;3:973-9. |
| 4. | Unüvar E, Oguz F, Sidal M, Kiliç A. Corticosteroid treatment of childhood Bell's palsy. Pediatr Neurol 1999;21:814-6. |
| 5. | Krishnamurthy S, Weinstock AL, Smith SH, Duffner PK. Facial palsy, an unusual presenting feature of childhood leukemia. Pediatr Neurol 2002;27:68-70. |
| 6. | Stein-Wexler R, Wootton-Gorges SL, West DC. Orbital granulocytic sarcoma: An unusual presentation of acute myelocytic leukemia. Pediatr Radiol 2003;33:136-9. |
| 7. | Baek HJ, Han DK, Kim YO, Choi IS, Hwang TJ, Kook H. Facial palsy as the presenting symptom of acute myeloid leukemia in children: Three cases with stem cell transplantations. Korean J Pediatr 2009;52:713-6. |
| 8. | Rohit K, Jitender MK, Atul S, Soumya S. The paradox of recurrent with rare: A rare case of bilateral proptosis and facial palsy in acute myeloid leukemia with recurrent cytogenetic translocation t(8:21). Int J Appl Basic Med Res 2015;5:76-8. |
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