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Journal of Pediatric Rehabilitation Medicine: An Interdisciplinary Approach 7 (2014) 267–272 DOI 10.3233/PRM-140295 IOS Press

Case Report

Rehabilitation outcomes after combined acute disseminated encephalomyelitis and Guillain-Barré syndrome in a child: A case report Radha Korupolua,∗, Thien Ngoa, Nawaz Hackb , Edward Escottc and Sara Sallesa a

Department of Physical Medicine & Rehabilitation, University of Kentucky, Lexington, KY, USA Center for Movement Disorders & Neurorestoration, University of Florida College of Medicine, Gainesville, FL, USA c Department of Radiology, University of Kentucky, Lexington, KY, USA b

Accepted 18 April 2014

Abstract. A 5-year old female presented with acute tetraparesis and areflexia. Initial imaging and cerebrospinal fluid analysis were suggestive of acute disseminated encephalomyelitis (ADEM). Minimal clinical response with intravenous steroids prompted further work up. Limited nerve conduction studies suggested possible acute motor-sensory axonal neuropathy, a rare variant of Guillain-Barré syndrome (GBS). Repeat imaging was compatible with polyradiculopathy indicating concomitance of ADEM and GBS. The patient suffered severe motor deficits and neuropathic pain. Slow but significant functional recovery was noted after intensive inpatient rehabilitation followed by continued rehabilitation via home health services. Keywords: Encephalomyelitis, Guillain-Barre syndrome, pediatrics, rehabilitation

OT PNS PT

Abbreviations ADEM AIDP CNS GBS IV IVIG MRI NCS

Acute disseminated encephalomyelitis Acute Inflammatory demyelinating polyneuropathy Central nervous system Guillain-Barré syndrome Intravenous Intravenous immunoglobulin Magnetic resonance imaging Nerve conduction studies

∗ Corresponding author: Radha Korupolu, Department of Physical Medicine & Rehabilitation, University of Kentucky, Lexington, KY, USA. E-mail: [email protected].

Occupational therapy Peripheral Nervous system Physical Therapy

1. Introduction Post infectious monophasic demyelinating disorders have been well defined in the literature and are often attributed to immune mediated inflammatory damage to the myelin sheath. Acute monophasic demyelination of the central nervous system (CNS) is known as acute disseminated encephalomyelitis whereas if this process is confined to the spinal cord it is recognized as transverse myelitis. If this acute process involves spinal nerve roots, cauda equina and/or peripheral nerves it

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R. Korupolu et al. / Rehabilitation outcomes after combined acute disseminated encephalomyelitis Table 1 Nerve conduction study data Nerve test Side & site Latency Amplitude

Median sensory Left palm 1.6 ms (normal) 17.1 µV(reduced)

Ulnar sensory Left palm 1.5 ms (normal) 9.9 µV (reduced)

Median motor Left wrist 2.3 ms (normal) 2.1 mV (reduced)

Median F-wave Left wrist 19.3 ms (normal) −

A

B

C

Fig. 1. A. Brain MRI: T2 weighted image of the brain shows patchy increased signal within both thalami. No abnormal enhancement was present. B. Cervical spine MRI: Sagittal STIR sequence shows mild spinal cord swelling and hazy diffuse increased signal. C. Axial T2 2D MEDIC (At the C4-5 level) shows patchy increased signal within the spinal cord, somewhat greater within the central gray, particularly the anterior horns.

is known as an Acute Inflammatory demyelinating polyneuropathy (AIDP) or typical Guillain-Barré syndrome (GBS) [1–3]. Several axonal variants of GBS exist which affects sensory and/or motor axons in peripheral nervous system (PNS) [1–3]. Usually either the CNS or the PNS is involved. However, concomitant or subsequent ADEM and GBS have been reported in the pediatric population [4–11]. We present a case report of the co-existence of ADEM and GBS resulting in severe debility in a 5 year old child. The patient’s detailed rehabilitation and recovery course presents an addition to the current published literature.

2. Case description A 5 year old previously healthy female presented to our acute care hospital with sudden onset of weakness in bilateral upper and lower extremities. She was diagnosed with a middle ear infection a week earlier and was treated with amoxicillin. Family reported sudden onset of weakness and pain in bilateral lower extremities on day 7 of amoxicillin. The patient was initially taken to the local emergency department and then subsequently, transferred to our acute care hospital. Neurological exam at admission revealed decreased alertness but oriented to self and parents, right eyelid ptosis,

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A

B

C

Fig. 2. MRI spine Day 21. A. Cervical spine MRI Sagittal STIR image shows that there is patchy increased signal within the spinal cord, although the signal and the swelling are somewhat improved. B. Lumbar spine MRI Sagittal T1 weighted post contrast image with fat saturation. There is extensive enhancement and thickening of the nerve roots of the cauda equina. C. Axial T-1 weighted post-contrast image shows that there is enhancement of cervical nerve roots.

left lateral nystagmus, paresthesia in lower extremities, flaccid tetraparesis affecting more prominently bilateral lower extremities and a generalized areflexia. The patient was voiding at the time of admission. At baseline, the patient had achieved normal developmental milestones.

3. Acute care hospital course On day 1 after admission, cerebrospinal fluid analysis revealed leukocytosis with a leukocyte count of 74 × 106 /L (4% neutrophils, 70% lymphocytes and 25% monocytes), elevated protein level (1.17 g/L), and elevated myelin basic protein level (10 µg/L). Magnetic resonance imaging (MRI) of the brain on day 2 showed (Fig. 1A) non-enhancing patchy areas of increased sig-

nal on T2 weighted images within the thalami and posterior aspect of the pons. MRI of the spine (Fig. 1B and 1C) indicated mild to moderate swelling of the spinal cord with non-enhancing intrinsic hyperintense signal on the T2-weighted images from C1 through the conus medullaris. These findings were consistent with ADEM. She was treated with 5 days of intravenous (IV) steroids which was started on day 3 soon after availability of MRI results. Minimal clinical response to IV steroids prompted treatment with intravenous immunoglobulin (IVIG) for 5 days which was started on day 8. This resulted in some improvement in her proximal upper and lower extremity motor strength. Subsequently, left upper extremity sensory and motor nerve conduction studies (NCS) were performed on day 18 due to persistent generalized areflexia. Sensory and motor responses from the left upper extremity showed

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R. Korupolu et al. / Rehabilitation outcomes after combined acute disseminated encephalomyelitis Table 2 Rehabilitation outcomes

Bed mobilitya Rolling Supine to sit Sit to supine Transfersa Sit to stand Bed to chair transfer Locomotiona Walking Wheel chair Balanceb Sitting Static Sitting Dynamic Standing Static Standing dynamic Activities of daily livinga Upper body dressing Lower body dressing Toileting Eating Grooming

Day 3 (Initial assessment at acute care)

Day 22 (IRH admission assessment)

Day 49 (IRH discharge assessment)

Day 105 (follow up)

Moderate assistance Maximum assistance Maximum assistance

Moderate assistance Maximum assistance Maximum assistance

Modified Independent Moderate assistance Moderate assistance

Independent supervision supervision

Total assistance Total assistance

Maximum assistance Total assistance

Moderate assistance Total assistance

Supervision Supervision

Unable to perform

Unable to perform

Unable to perform

Total assistance

Supervision (50 ft in a gait trainer) Supervision (> 150 ft)

Supervision (101–125 ft with walker) −

Poor Unable to balance n/a n/a

Poor Poor n/a n/a

Fair Fair Poor Poor

Good Good Fair Fair

Total assistance Total assistance Total assistance Moderate assistance Total assistance

Total assistance Total assistance Total assistance Moderate assistance Total assistance

Minimum assistance Minimum assistance Total assistance Modified independent −

− − − − −

a Evaluated

using the Wee Functional Independent measure scoring system. (Ref: Clinical Pediatrics(Phila). 1994 Jul; 33(7): 431-8); b Evaluated using the functional balance grades (Ref: O’Sullivan, S.B. and Schmitz T.J. (2007). Physical rehabilitation: assessment and treatment 5th ed.). c On day 105 unable to report activities of daily living due to lack of formal assessment by occupational therapist. d Unable to report wheel chair status on day 105 due to missing documentation by physical therapist.

decreased amplitude with normal latency and normal F wave latency (Table 1). These findings were suggestive of possible acute motor-sensory axonal neuropathy, a rare variant of GBS. The patient was unable to tolerate further NCS and needle electromyography secondary to pain and the procedure was aborted after the left upper extremity NCS. Repeat MRI of the brain on Day 21 indicated essentially resolution of the brain parenchymal lesions relative to the prior studies. New enhancement was noted involving the 3rd cranial nerves, and probably the cranial nerves within the internal auditory canals, on the post contrast series. MRI of the spine showed some improvement in previously seen spinal cord swelling and abnormal signal (Fig. 2A), but now with pronounced enhancement of multiple cervical and thoracic spinal nerve roots and of the cauda equina (Fig. 2B and 2C). These findings along with NCS findings were compatible with a diagnosis of GBS suggesting concomitance of ADEM and GBS. Results of extensive viral and bacterial serologic tests were negative for infection. Physical therapy (PT) and occupational therapy (OT) were initiated on day 4 in the acute care hospital. The patient required aggressive bowel care on day 3 in the acute care hospital secondary to constipation. She was

started on scheduled osmotic laxative for management of bowels which was later discontinued prior to discharge due to multiple loose stools. The patient was able to empty bladder without requiring catheterization. Post void residuals were within normal range for age. However, the patient was using diapers in acute care hospital as a result we cannot rule out urinary incontinence. The patient was discharged to our inpatient rehabilitation facility on day 22 from acute care hospital.

4. Inpatient rehabilitation facility course On admission to the inpatient rehabilitation facility, the patient exhibited severe neuropathic pain in all extremities and persistent tetraparesis. She was continent of bowel and bladder. Post void residuals remained within acceptable range for age. The patient was able to recognize and communicate when she needed to empty her bowels and bladder. She required moderate to maximum assistance for bed mobility and total assistance for bed to chair transfers and activities of daily living (Table 2). She had poor sitting balance requiring

R. Korupolu et al. / Rehabilitation outcomes after combined acute disseminated encephalomyelitis

total assistance for sitting upright. She was unable to ambulate. During a 27-day inpatient rehabilitation stay, the patient received intensive physical and occupational therapy to maximize functional recovery. Gabapentin was started for neuropathic pain with gradual titration of dose. Ankle foot orthoses were prescribed for bilateral foot drop. At discharge, she was able to ambulate 50 feet in a gait trainer and commando crawl up to 20 feet with supervision. Significant improvement was noted in her ability to propel her wheelchair for a distance of greater than 150 feet with supervision. Her sitting balance was fair by the time of discharge and was significantly improved from admission. She was able to perform upper body and lower body dressing with minimal assistance. She was discharged to home with continued rehabilitation services via home health. Adaptive equipment recommendations at discharge included booster car seat, manual wheelchair, pediatric walker, standing frame, pediatric bedside commode and a tub seat with chest strap.

5. Follow up On follow up 2 months after discharge from our inpatient rehabilitation facility and review of home health PT/OT records, continued progress in her functional ability and balance was noted. She was able to ambulate with a walker up to 125 feet with supervision. Her sitting balance was good and standing balance was fair. Her neuropathic pain was controlled on gabapentin without requiring any increase in dose since her discharge from the inpatient rehabilitation. On recent follow up 6 months after discharge from inpatient rehabilitation, the patient was able to ambulate with bilateral ankle foot orthoses without the use of an assistive device at community level ambulation for her age. She was independent with bed mobility, transfers and activities of daily living with exception of supervision for bathing.

6. Discussion ADEM and GBS typically occurs in the pediatric population after a viral-induced autoimmune response [11]. The combination of these two diseases causes a broad spectrum of neuromuscular abnormalities including, but not limited to, altered consciousness, areflexia or hyperreflexia, hypotonia or hyper-

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tonia, sensory disturbances, neurogenic bowel, neurogenic bladder, autonomic dysfunction and generalized weakness [4–11]. Cerebrospinal fluid analysis, MRI and, electrodiagnostic studies have been frequently used to confirm the diagnosis [4–11]. Acute treatment for this combined condition (ADEM and GBS) can include IVIG, IV high dose corticosteroids, and sometimes plasma exchange [4–11]. A number of case reports and a case series reported favorable recovery with some mild residual deficits during follow up period ranging from 2 months to 1 year in combined GBS and ADEM or transverse myelitis [4–6,8,9]. However, in three retrospective studies overall poorer outcomes were reported in combined ADEM and GBS during follow up compared to isolated ADEM or GBS [7,10,11]. In one retrospective study, 13 patients (age range 1 to 17 years) were identified with both ADEM and GBS [7]. During a median follow up of 9.2 months, 7 patients (54%) remained severely disabled, mobility restricted to bed or wheelchair, and/or mechanical ventilatordependent [7]. In another retrospective study, 8 patients (age range 2 to16 years) suffering from simultaneous polyradiculoneuritis and myelitis were identified [10]. During a median follow up period of 11.5 months, 7 patients (88%) suffered from residual paraparesis or monoparesis of a lower limb [10]. The other recently published retrospective study reported outcomes of 5 patients (age range 8 to 15 years) with concurrent acute myelitis and GBS [11]. At a follow-up of 1 year or longer, one patient recovered, and four patients remained with residual paresis. The mean disability score in the group with concurrent acute myelitis and GBS indicated significantly poorer functional status than in the group with GBS alone [11]. Our patient received appropriate early medical and rehabilitation therapy followed by approximately 4 weeks of intensive rehabilitation therapy under the care of a multidisciplinary team. Significant functional improvement was achieved at discharge from our inpatient rehabilitation facility with continued recovery noted at follow up. In conclusion, limited evidence in the literature reports prolonged and incomplete recovery in children after combined ADEM and GBS. Prompt diagnosis of this combined condition is important to initiate timely aggressive medical interventions and early involvement of rehabilitation services which may expedite the recovery process resulting in better functional outcomes. Multidisciplinary team work, early and intensive inpatient rehabilitation, and continuum of care ap-

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pear to be critical in such cases. This case report was limited by the lack of further NCS studies of other limbs involved and also by the lack of EMG data due to patient discomfort and subsequent refusal to continue the study by her parents. However, our physical exam findings along with brain and spinal cord imaging strongly support simultaneous central and peripheral nervous system involvement.

[3]

[4]

[5]

[6]

Acknowledgement/Conflict of interest Escott, E. J. has following financial disclosures: Royalties, Thieme Medical Publishers, Inc Researcher, Athersys, Inc Grant, Athersys, Inc.

[7]

[8]

[9]

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Rehabilitation outcomes after combined acute disseminated encephalomyelitis and Guillain-Barré syndrome in a child: a case report.

A 5-year old female presented with acute tetraparesis and areflexia. Initial imaging and cerebrospinal fluid analysis were suggestive of acute dissemi...
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