Annals of Tropical Medicine and Public Health

CASE REPORT
Year
: 2017  |  Volume : 10  |  Issue : 6  |  Page : 1824--1826

Motor axonal neuropathy in sequence with acute transverse myelitis


Smita Nath, Pinkesh Parmar, V Prabhu, Sandeep Garg, Suresh Kumar 
 Departments of Medicine, Maulana Azad Medical College and Lok Nayak Hospital, New Delhi, India

Correspondence Address:
Smita Nath
Flat No. 1, Ajanta Appartment 36 IP Extension, Patparganj, New  Delhi - 110  092
India

Abstract

Acute transverse myelitis (ATM) is an upper motor neuron disease of the spinal cord, and its association with peripheral polyneuropathy, particularly the axonal type, is rarely reported. This case presented with features of noncompressive upper motor neuron type of quadriplegia with bladder, bowel and sensory involvement at C4 spinal level. The diagnosis of ATM was confirmed by magnetic resonance imaging scan. The patient responded to high-dose steroids and was discharged. However, after 2 weeks of recovery, the patient again developed acute-onset pure motor flaccid paralysis. Nerve conduction test was diagnostic of pure motor axonal neuropathy. This time she was treated with immunoglobulins after which she improved.



How to cite this article:
Nath S, Parmar P, Prabhu V, Garg S, Kumar S. Motor axonal neuropathy in sequence with acute transverse myelitis.Ann Trop Med Public Health 2017;10:1824-1826


How to cite this URL:
Nath S, Parmar P, Prabhu V, Garg S, Kumar S. Motor axonal neuropathy in sequence with acute transverse myelitis. Ann Trop Med Public Health [serial online] 2017 [cited 2019 Dec 15 ];10:1824-1826
Available from: http://www.atmph.org/text.asp?2017/10/6/1824/222661


Full Text



 Introduction



Transverse myelitis is an acute or subacute disorder that induces segmental demyelination or necrosis at several spinal cord levels.[1] Patients with acute transverse myelitis (ATM) present with pain, fever, and a combination of motor, sensory, and sphincter dysfunctions thought to be the result from an autoimmune response, which may be idiopathic, or may be associated with postinfectious and parainfectious phenomena.[1],[2] Guillain-Barre syndrome (GBS) is an acute inflammatory peripheral polyneuropathy consisting of mainly four subtypes. Typically, acute inflammatory demyelinating polyradiculoneuropathy is observed in the form of ascending symmetrical weakness and areflexia due to postinfectious autoimmune etiology resulting in molecular mimicry.[3],[4] ATM is generally an upper motor neuron disease of the spinal cord while GBS is lower motor neuron disease. We present a case where ATM was followed by pure motor axonal type polyneuropathy.

 Case Report



A 40-year-old female patient was admitted to emergency with insidious onset progressive quadriplegia of 1-week duration associated with bowel and bladder involvement from the onset. She had no history of fever, diarrhea, and upper respiratory tract infection in the recent past. On examination, the patient was afebrile, with pulse of 100/min, blood pressure of 100/70 mmHg, and respiratory rate of 24/min. There was no pallor, icterus, lymphadenopathy, thyromegaly, cyanosis, and clubbing, and Jugular venous pressure was not raised. Central nervous system (CNS) examination revealed no deficiency in higher mental and cranial nerve functions. Power in bilateral upper and lower limbs was 1/5 with preserved muscle bulk and no fasciculations. Her deep tendon reflexes (DTRs) were exaggerated and plantar reflex was extensor bilaterally. There was complete sensory loss of all modalities till C4 spinal level along with urinary retention. Examination of skull and spine was within normal limits. There was no respiratory muscle weakness. Rest of the systemic examination was normal. On investigation, hemoglobin (Hb) was 10 gm%, total leukocyte count (TLC) - 6,100, platelets 2.75 lakh, blood urea - 24, serum creatinine - 0.4, s. bilirubin - 0.6, SGOT-18 IU/L, SGPT-23IU/L, serum protein - 6 gm/dl, albumin - 3 gm/dl, erythrocyte sedimentation rate (ESR) - 48, serum calcium - 8.5 gm/dl, uric acid - 4.5 gm/dl, lactate dehydrogenase (LDH) - 100 mg/dl, and creatine phosphokinase CPK- 54U/L. Blood and urine cultures were negative. All the hematological and biochemical parameters were normal. A provisional diagnosis of noncompressive upper motor neuron type of quadriplegia with bowel bladder and sensory involvement probably ATM was made. Magnetic resonance imaging (MRI) of cervical spine with screening of whole spine on the day 3 of admission revealed symmetrical T2 hyperintensities in medulla (in region of corticospinal tract) and subtle T2 hyperintensities in cervical cord likely ATM [Figure 1]. MRI brain was normal. The patient was started on high-dose methylprednisolone (1 g/day) for 3 days followed by oral steroids subsequently. The patient showed recovery over following weeks with the treatment. Her motor power improved along with bladder function.{Figure 1}

After 2 weeks of discharge, she was readmitted with complaints of acute-onset ascending quadriparesis of 3 days duration. However, there was no deterioration in sensory modalities and bowel bladder function. CNS examination at this time showed power of 0/5 in B/L upper and lower limbs. DTR were absent and plantar reflexes were mute. On investigation this time, Hb was 11.1 gm%, TLC - 7,700, platelets - 2.75 lakh, blood urea - 19 gm/dl, serum creatinine - 0.5 gm/dl, serum bilirubin - 0.4, SGOT - 41, SGPT – 21, serum protein - 5.4 gm/dl, albumin - 3 gm/dl, ESR - 40, serum calcium - 8.7 gm/dl, serum uric acid - 4.5 gm/dl, LDH - 150 mg/dl, and CPK (T) - 66 mg/dl. All the hematological and biochemical profile was normal. Cerebrospinal fluid (CSF) was acellular with a protein level of 160 and sugar of 64 mg/dl. CSF viral serology for herpes simplex virus was negative. Antinuclear antibody was negative. A provisional diagnosis of acute demyelinating polyradiculopathy was made. Nerve conduction study revealed pure motor primary axonal polyneuroradiculopathy. The patient was started on intravenous immunoglobulin at dose of 2 g/kg over 5 days. The patient showed improvement to the treatment.

 Discussion



Transverse myelitis is noncompressive and mostly demyelinating affliction of the spinal cord characterized by symptoms and signs of neurologic dysfunction in its motor and sensory pathway. There may be a history of viral or febrile illness in the weeks preceding the illness.[2] It is believed to be a demyelinating illness and its most common cause includes infections primarily viral, spinal cord infarction, autoimmune disorders such as systemic lupus erythematosus (SLE), sarcoidosis, demyelinating diseases such as multiple sclerosis and neuromyelitis optica or idiopathic transverse myelitis.[1],[2]

On the other hand, GBS is an acute and often fulminant polyradiculopathy with an underlying autoimmune etiology. Its incidence is 1–4 cases per 100,000 out of which approximately 70% cases occur within 1–3 weeks of an acute infectious process, usually respiratory or gastrointestinal.[3],[4]Campylobacter jejuni causes 20%–30% of these cases whereas human herpes virus, cytomegalovirus, and Epstein–Barr virus account for another 20%–30%. Other viruses such as HIV, Hepatitis E and Mycoplasma pneumoniae have been identified as causative agents.[5],[6] GBS is also seen in lymphoma and SLE.[4],[11] Substantial evidence supports an autoimmune basis for all the subtypes of GBS resulting from immune response to non-self-antigens (infectious agents and vaccines) through molecular mimicry. Antiganglioside antibodies most frequently to GM1 are common in GBS (20%–50%) particularly in acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy variety. Cases preceded by C. jejuni infection has caused epidemic of AMAN variety of GBS among children and young adults in China and Mexico. Pathologically nodes of Ranvier are first to bear the brunt of initial insult and the extent of axonal damage is highly variable.[3],[4]

Although concurrency of GBS and ATM is rare, transitions between demyelinating diseases such as GBS and ATM are possible. For the first time in 2001, a case of ATM and GBS occurring after a mumps virus infection was published by Bajaj et al.[7] Acute mumps viremia was confirmed on serological grounds in this case. Until now, 7 cases of concurrencies of GBS and ATM have been documented in literature. Out of seven, six cases were published after 2007.[10] It was found in the literature that the responsible organisms in etiology of concurrent GBS and ATM are mycoplasma, influenza, Bartonella henselae, and mumps. The other postulated etiology is autoimmunity.[10],[12] In 2011, Bonastre-Blanco et al. describe the case of a 14-year-old female patient who was diagnosed as case of myositis along with transverse myelitis, and GBS, caused by M. pneumoniae infection.[8] A similar case was reported by Topcu et al.[11] Carman et al. in 2013 described the coexistence of ATM and GBS in a 12-year-old boy with Bartonella infection.[9] Howell et al. in 2007 reported a case of a 14-year-old boy who presented with acute paraparesis with sensory and sphincter disturbance. Imaging and neurophysiologic studies were diagnostic for transverse myelitis with AMAN.[10] As in our case, the etiology was not established in this case also.[10] Such dual lower and upper motor neuron pathology may be associated with a poorer outcome and indicates simultaneous central and peripheral immune-mediated injury.[10] The above case reports clearly underline the presence of autoimmune or an infectious cause behind the concurrence of two demyelinating entities in the same patient. Most of these cases were reported in pediatric age group which is different to our case where the same thing has happened in an adult female.

 Conclusion



Two demyelinating diseases such as GBS and ATM can be observed together in the patient either simultaneously or in succession indicating both the central and peripheral involvement which may get involved subsequently in the due course of the disease. It should be taken into account in those cases who do not respond to therapies or whose recovery takes more time than anticipated.

 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.

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