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CASE REPORT Table of Contents  
Ahead of print publication
Cranial polyneuropathy and polyradiculopathy with intractable vomiting due to a rare etiology


1 Department of Neurology, National Institute of Mental Health and Neurosciences, Indiaeurosciences, Bengaluru, Karnataka, India
2 Department of Neuropathology, National Institute of Mental Health and Neurosciences, Indiaeurosciences, Bengaluru, Karnataka, India

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Date of Submission16-Mar-2022
Date of Decision29-Mar-2022
Date of Acceptance03-May-2022
Date of Web Publication21-Sep-2022
 

  Abstract 

Multiaxial involvement in neurological cases poses a diagnostic challenge even to the astute clinician. We present the case of a young man with multiple cranial nerve palsies, brain stem involvement, and subacutely progressive polyradiculoneuropathy. In addition, he had unexplained intractable vomiting, lymphadenopathy, and splenomegaly. These atypical features prompted us to evaluate for a secondary or systemic cause of polyneuropathy. Evaluation with neuroimaging and bone marrow examination revealed the underlying cause to be pre-T-cell acute lymphoblastic leukemia (pre-T-ALL). While acute lymphoblastic leukemia (ALL) is known to cause central nervous system involvement, such extensive multiaxial involvement as the first presentation in an adult is extremely rare. In this report, we describe the diagnostic approach to such a case and review the literature.

Keywords: Acute T-cell leukemia, cranial nerve palsies, subacute polyradiculoneuropathy


How to cite this URL:
Koshy KG, Taallapalli A V, Rajula RR, Borse PR, Nashi S, Nandeesh BN, Kulkarni GB. Cranial polyneuropathy and polyradiculopathy with intractable vomiting due to a rare etiology. APIK J Int Med [Epub ahead of print] [cited 2022 Sep 25]. Available from: https://www.ajim.in/preprintarticle.asp?id=356503



  Introduction Top


A fundamental axiom in clinical neurology states that symptoms and signs should be attributed to a single cause whenever possible. Hence, patients presenting with the involvement of multiple axes of the central and peripheral nervous systems pose a diagnostic challenge. A patient with polyradiculoneuropathy can be categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), subacute inflammatory demyelinating polyradiculoneuropathy (SIDP), or chronic inflammatory demyelinating polyradiculoneuropathy based on the duration of the evolution of symptoms. In each case, secondary causes must be ruled out. Leukemias and lymphomas are known to present with symptoms in various areas of neuraxis due to infiltration in the nervous system. However, an adult patient presenting for the first time with symmetric cranial neuropathies, brain stem involvement, and polyradiculopathy is unusual. In this case report, we describe a patient presenting with intractable vomiting, multiple cranial nerve palsies, and polyradiculopathy due to T-cell acute lymphocytic leukemia (T-ALL) and briefly review the literature and approach to such a patient.


  Case Report Top


A previously healthy 21-year-old man presented with recurrent episodes of vomiting for 2 months. Three days after the onset of the first symptom, he noticed difficulty in closing both eyes. One week after the beginning of the illness, he developed progressive difficulty in smiling and nasal regurgitation on swallowing liquids. Six weeks into the disease, he noticed buckling of the left knee and became bedbound in 2 days. His upper limbs, bowel, and bladder functions remained normal. Over the last 2 months, he had lost 10 kg of weight.

On examination, he was emaciated. He had enlarged, firm, and nontender posterior cervical lymph nodes on both sides; the largest one was 2 cm × 2 cm in size. The spleen was palpable 2 cm below the left costal margin, firm, and nontender. Bilateral sixth, lower motor neuron type, seventh, ninth, and tenth nerve palsies were present. The upper extremities were hypotonic and areflexic with normal power (5/5). The lower limbs were hypotonic, areflexic, and weak (Grade 2–3/5). The neck flexors (Grade 2/5) and truncal muscles were also weak.

A diagnosis of SIDP was considered. However, vomiting, weight loss, lymphadenopathy, splenomegaly, and descending pattern of weakness were unusual for SIDP. Blood investigations showed leukocytosis (12500 cells/mm3 with 41% neutrophils, 45% lymphocytes, and 12% monocytes) without abnormal cells. Nerve conduction studies were normal except for absent F waves in both common peroneal nerves and impersistent in bilateral median, ulnar, and tibial nerves.

Contrast-enhanced magnetic resonance imaging (MRI) of the brain and spinal cord showed diffuse thickening and enhancement of the bilateral third, fifth, sixth, seventh-eighth complex, ninth-tenth-9th complex, and 12th nerves [Figure 1]a with an enhancing nodular lesion in the dorsal medulla [Figure 1]b. There was a diffuse leptomeningeal enhancement over the spinal cord [Figure 1]c with the enhancement of the cauda equina [Figure 1]d. Esophagogastroduodenoscopy was normal. Serology for human immunodeficiency virus was negative. Lumbar puncture yielded a dry tap. Given the MRI findings, the provisional diagnosis was revised to acute polyradiculopathy secondary to a diffuse infiltrative cause. Computed tomography of the chest and abdomen showed extensive cervical, mediastinal, and abdominal lymphadenopathy with splenomegaly. The largest node was seen in the anterior mediastinum [Figure 2]a. In addition, both kidneys were enlarged [Figure 2]b. Bone marrow aspiration showed 52% blast cells. Flow cytometry showed features of Pre-T ALL [Table 1]. He was referred to an oncologist for further management.
Figure 1: (a) MRI brain (T1 contrast, axial image) shows bilateral enhancing facial nerves (arrows); (b) MRI brain (T1 contrast, axial image) shows enhancing lesion in the dorsal medulla (red arrow) The red arrow in panel b suggests enhancement in dorsal medulla; which occurs due to malignancy invading cerebral parenchyma; (c) MRI cervical spine (T1 contrast, sagittal image) reveals leptomeningeal enhancement; (d) MRI lumbosacral spine (T1 contrast, sagittal image) reveals enhancement of cauda equina. MRI: Magnetic resonance imaging

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Figure 2: (a) CECT chest shows enlarged anterior mediastinal lymph node (red arrow); (b) CECT abdomen reveals bilateral renomegaly. CECT: Contrast-enhanced computed tomography

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Table 1: Flow cytometry showing markers suggestive of pre-T cell acute lymphoblastic leukemia (CD1a, CD2, CD3, CD4, CD5, CD7, CD8, and aberrant CD10 positive)

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  Discussion Top


We describe a case of a young male who presented with complaints of subacute onset symmetric cranial polyneuropathy and polyradiculopathy with significant weight loss. The evaluation showed leukocytosis, lymphadenopathy, splenomegaly, renomegaly, and blast cells in the bone marrow. The final diagnosis was pre-T ALL.

Leukemias and lymphomas are disorders characterized by the uncontrolled proliferation of malignant blood cells. These can involve neuraxis by diffuse meningeal infiltration, intra-axial or extra-axial mass lesions compressing the brain, spinal cord, or peripheral nerves by vascular occlusions secondary to direct invasion by tumor cells hypercoagulability or by causing paraneoplastic syndromes.[1] Structural mass lesions are common in lymphomas, followed by myeloid leukemias (chloroma). Leptomeningeal infiltration is common in non-Hodgkin's lymphoma.[2] Cranial nerve palsies and peripheral nerve root involvement can occur due to infiltration.

Central nervous system (CNS) involvement in leukemia is most common in acute lymphatic leukemia (ALL).[3] Up to 50% of ALL cases may have CNS involvement at some point in the disease course. However, ALL presenting for the first time with CNS manifestations occurs in <5% of the cases.[4] Cerebrospinal fluid cytology or stereotactic brain biopsy may be helpful for a definitive diagnosis of CNS involvement by leukemia. In such cases, in addition to standard treatment for ALL, intrathecal chemotherapy with cytarabine, methotrexate, and cranial irradiation are advised.[5]

Acute leukemia presenting for the first time as cranial neuropathy is uncommon. Bifacial palsy as a presenting feature has been described earlier.[6],[7]

In our case, the patient initially had bilateral abducens facial and bulbar palsy. Given the cranial nerve enhancement on MRI, this may be due to direct nerve infiltration. The flaccid lower limb weakness with generalized areflexia was likely due to polyradiculitis secondary to tumor infiltration. The intractable vomiting may be due to the medullary lesion. The presence of a sizeable mediastinal lymph node was a clue to the diagnosis of T-cell ALL in this case. In a case series of acute T-lymphoblastic leukemia from India, it was found that 53% had mediastinal lymphadenopathy.[8]

The secondary causes of polyradiculopathy include porphyria, neoplastic infiltration, paraneoplastic syndrome, arachnoiditis, sarcoidosis, vasculitis, and Lyme disease. The presence of systemic symptoms favors a secondary cause.


  Conclusion Top


Patients presenting with symptoms in multiple axes pose a significant challenge for diagnosis. The presence of systemic symptoms and signs of polyradiculopathy is a clue for a secondary cause. Hence, an extensive investigation has to be carried out.

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.
Glass J. Neurologic complications of lymphoma and leukemia. Semin Oncol 2006;33:342-7.  Back to cited text no. 1
    
2.
Yoshida S, Morii K, Watanabe M, Saito T. Characteristic features of malignant lymphoma with central nervous system involvement. Surg Neurol 2000;53:163-7.  Back to cited text no. 2
    
3.
Rodriguez-Abreu D, Bordoni A, Zucca E. Epidemiology of hematological malignancies. Ann Oncol 2007;18 Suppl 1:i3-8.  Back to cited text no. 3
    
4.
Barredo J, Ritchey AK. Controversies in the management of central nervous system leukemia. Pediatr Hematol Oncol 2010;27:329-32.  Back to cited text no. 4
    
5.
Thomas X, Le QH. Central Nervous system involvement in adult acute lymphoblastic leukemia. Hematology 2008;13:293-302.  Back to cited text no. 5
    
6.
Koury JC. Acute lymphoblastic T cell leukemia restricted to the central nervous system: A case report. J Blood Disord Treat 2019;2:1-4.  Back to cited text no. 6
    
7.
Sen S, Gupta A, Friedman P, Naina HV. Bilateral facial nerve palsy in acute B cell lymphoblastic leukemia: A case report and review of the literature. Indian J Hematol Blood Transfus 2016;32 Suppl 1:15-9.  Back to cited text no. 7
    
8.
Mathan LP, Ananthamurthy A. Clinicopathological attributes of T-lymphoblastic lymphoma seen in a tertiary care center. Clin Cancer Investig J 2018;7:1-8.  Back to cited text no. 8
  [Full text]  

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Correspondence Address:
Girish Baburao Kulkarni,
Department of Neurology, National Institute of Mental Health and Neurosciences, Hosur Road, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ajim.ajim_37_22



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    -  Koshy KG
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    -  Nashi S
    -  Nandeesh BN
    -  Kulkarni GB


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