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Table of Contents
LETTER TO THE EDITOR
Year : 2013  |  Volume : 16  |  Issue : 3  |  Page : 463-464
 

Symptomatic trigeminal neuralgia as the first presentation of acute lymphoblastic leukemia


1 Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Radiotherapy, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India
3 Department of Pathology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Date of Web Publication26-Aug-2013

Correspondence Address:
Tamojit Chaudhuri
Department of Radiotherapy, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow 226 014, Uttar Pradesh, Lucknow 226 014, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-2327.116962

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How to cite this article:
Chaudhuri T, Mondal D, Yadava K. Symptomatic trigeminal neuralgia as the first presentation of acute lymphoblastic leukemia. Ann Indian Acad Neurol 2013;16:463-4

How to cite this URL:
Chaudhuri T, Mondal D, Yadava K. Symptomatic trigeminal neuralgia as the first presentation of acute lymphoblastic leukemia. Ann Indian Acad Neurol [serial online] 2013 [cited 2019 Jun 24];16:463-4. Available from: http://www.annalsofian.org/text.asp?2013/16/3/463/116962


Sir,

It is not unusual for the cranial nerves to be affected early in the course of acute lymphoblastic leukemia (ALL) due to the meningeal infiltration. Here, we report a 19-year-old male with the symptomatic trigeminal neuralgia and right third, fourth and sixth cranial nerve palsy as initial presentation of high-risk T-cell ALL. To the best of our knowledge, symptomatic trigeminal neuralgia as the first presentation of ALL has not been reported earlier.

A 19-year-old Asian male presented with complaints of painful sharp electric shock-like transient spasms on the right hemi-face around the eye and cheek for last 2 months and diplopia for last 15 days. The painful spasms usually lasted for a few seconds to minutes and triggered by light touch and activities such as brushing teeth, talking, and blowing his nose. On examination, right third, fourth and sixth cranial nerve palsy was found. The rest of the general and neurological examination was normal, including trigeminal sensations. Magnetic resonance imaging (MRI) of the brain showed a right parasellar mass, which was hyperintense on T1, isointense on T2 and fluid attenuated inversion recovery sequences; and intensely contrast enhancing on T1 sequence with the gadolinium contrast [Figure 1]. Involvement of right cavernous sinus and encasement of ipsilateral internal carotid artery were also evident from the MRI. Peripheral blood picture and bone marrow examination revealed features consistent with ALL. Cerebrospinal fluid cytology revealed the presence of leukemic blasts. Thyroid function tests, antinuclear factor, serum immunoglobulins, immunoelectrophoresis, and antibodies to acetylcholine receptor were all within normal limits. Immunophenotyping showed cluster of differentiation (CD) 3 and CD5 positivity. In view of positivity for chromosomal translocation t (4; 11) (q21; q23), he was diagnosed as a case of high-risk T-cell ALL, presenting as a paraseller mass (leukemic deposit) with right sided symptomatic trigeminal neuralgia and third, fourth and sixth cranial nerve palsy. For trigeminal neuralgia, the patient was recommended carbamazepine, 800 mg daily. He was given chemotherapy as per ALL-Berlin-Frankfurt-Munster 95 protocol. At first remission, he received 18 gray/10 fractions therapeutic cranial irradiation, followed by 8 gray/4 fractions boost to the right parasellar mass with 3-dimentional conformal radiotherapy technique. After 3 months of treatment completion, there was partial improvement of diplopia and the trigeminal neuralgia was well-controlled.
Figure 1: Magnetic resonance imaging of the brain showing a T1 hyperintense, T2 isointense right parasellar mass with involvement of right cavernous sinus, and encasement of ipsilateral internal carotid artery

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Cranial nerve palsies as the first presentation of ALL, although uncommon, but have already been reported. [1],[2],[3] There have been also some reports of cranial nerve palsies as the first sign of relapse or recurrence in ALL patients. [4],[5] Unless specifically treated, the neurological deficit is usually progressive in this situation. We believe that this is the first description of a patient with a systemic lymphoproliferative disorder, namely ALL, presenting with symptomatic trigeminal neuralgia. The cause of the right third, fourth and sixth cranial nerve palsies in our patient was most probably leukemic involvement of the right cavernous sinus, as was shown by MRI. There was partial improvement of the motor functions of the right eye, even after 3 months of treatment completion. The probable causes might be late presentation with some irreversible ischaemic damage to the cranial nerves due to the pressure effect of the right parasellar leukemic deposit and delay in radiotherapy.

To conclude, symptomatic trigeminal neuralgia and cranial nerve palsies led to the diagnosis of ALL in this patient with minimal systemic clues.

 
   References Top

1.Oz O, Akgün H, Battal B, Yücel M, Oztürk K, Karadurmuº N, et al . Acute lymphoblastic leukemia presenting with oculomotor nerve palsy. Acta Neurol Belg 2011;111:362-4.  Back to cited text no. 1
    
2.Steele JA, Hull D. Acute lymphoblastic leukaemia presenting with an isolated sixth cranial nerve palsy in an adult. Eur J Emerg Med 1994;1:203-4.  Back to cited text no. 2
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3.Schattner A, Kozack N, Sandler A, Shtalrid M. Facial diplegia as the presenting manifestation of acute lymphoblastic leukemia. Mt Sinai J Med 2001;68:406-9.  Back to cited text no. 3
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4.Schwartz CL, Miller NR, Wharam MD, Leventhal BG. The optic nerve as the site of initial relapse in childhood acute lymphoblastic leukemia. Cancer 1989;63:1616-20.  Back to cited text no. 4
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5.Harada Y, Kuriyagawa K, Okada K, Shirota T, Ito H. Anterior segment of the eye and cranial nervous (II, III, VI, VII) infiltration in relapsing acute lymphoblastic leukemia (L1). Rinsho Ketsueki 1994;35:1371-7.  Back to cited text no. 5
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