|Year : 2019 | Volume
| Issue : 1 | Page : 45-47
A case of flaccid quadriparesis
Kashish Gupta, Subhash Kumar, Narendra Bishnoi, Mayank Gupta, Sunil Kumar Mahavar, Raman Sharma
Department of Internal Medicine, SMS Medical College, Jaipur, Rajasthan, India
|Date of Submission||24-Sep-2018|
|Date of Decision||31-Oct-2018|
|Date of Acceptance||20-Dec-2018|
|Date of Web Publication||18-Feb-2019|
Dr. Mayank Gupta
S-81, Hardik Medicos, Barkat Nagar Chouraha, Tonk Phatak, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
Inclusion body myositis (IBM) is the most common inflammatory myopathy above the age of 50 years and three times more common in males than females. It presents as a distal more than proximal myopathy and has an indolent progressive course. Despite the latest advancements, it is challenging to diagnose this disease as it may resemble amyotrophic lateral sclerosis (ALS) clinically and polymyositis histopathologically. What sets it apart from the other myopathies is the fact that it has a very poor response to standard therapies of steroids and immunosuppressants. We present a case of a 30-year-old female patient presenting with relatively rapid onset of quadriparesis and dysphagia which was ultimately diagnosed with IBM. This case report attempts to highlight the difficulties in diagnosing this rare disease and the limited modalities of treatment.
Keywords: Inclusion body myositis, inflammatory myopathy, quadriparesis
|How to cite this article:|
Gupta K, Kumar S, Bishnoi N, Gupta M, Mahavar SK, Sharma R. A case of flaccid quadriparesis. Indian J Med Spec 2019;10:45-7
|How to cite this URL:|
Gupta K, Kumar S, Bishnoi N, Gupta M, Mahavar SK, Sharma R. A case of flaccid quadriparesis. Indian J Med Spec [serial online] 2019 [cited 2019 May 24];10:45-7. Available from: http://www.ijms.in/text.asp?2019/10/1/45/252470
| Introduction|| |
The inflammatory myopathies represent the largest group of acquired and potentially treatable causes of skeletal muscle weakness. On the basis of distinct clinicopathological features, they can be divided into four subtypes: polymyositis (PM), Dermatomyositis (DM), Inclusion body myositis (IBM), Necrotizing autoimmune myositis.
The prevalence of inflammatory myopathies is estimated at 1 in 100,000. IBM remains a poorly understood form of idiopathic inflammatory myopathy; although, great progress in the areas of clinical recognition and pathophysiology have been made recently. The progressively worsening muscle weakness may sometimes be misdiagnosed with treatment-resistant PM. IBM also may be misdiagnosed as ALS (ALS or Lou Gehrig's disease).
Here, we present a rare case of IBM. This clinical report highlights the difficulties in diagnosing the disease and the poor response to treatment.
| Case Report|| |
Thepatient was 30-year-old, right-handed, Hindu, married female, a resident of Dausa (Rajasthan), housewife and educated up to XII class. She presented with the chief complaints of fever for 45 days with acute-onset weakness in both lower limbs from the last 30 days followed by weakness in both upper limbs from 20 days. She also complained of difficulty in swallowing solids and liquids from the last 7 days. Fever was insidious in onset, low grade, not associated with chills and rigors, rash. It was relieved by taking medication. Patient apparently normal 30 days back, developed sudden-onset weakness of both lower limbs which was noticed as difficulty in standing up from squatting position which progressed in a matter of 10 days to involve both upper limbs in form of difficulty in holding glass of water, buttoning and unbuttoning clothes, breaking chapatis, and slippage of slippers. Her weakness was not associated with any diurnal variation. Over the last 7 days, she also developed difficulty in swallowing semi-solid food, and had a choking sensation on deglutition. There was no nasal regurgitation.
Her weakness progressed to involve the neck muscles and neck had to be supported when erect. There was no history of difficulty in chewing, vision, drooping of eyelids, facial muscle weakness, tinnitus, hoarseness of voice, and involuntary movements, bowel or bladder involvement, backache, radicular or neck pain, band-like sensation, sore throat, tingling numbness, recent vaccination, or unknown bite. Past, personal, and family history was noncontributing.
On examination, the patient was averagely built and poorly nourished. She was afebrile at presentation with higher mental functions and cranial nerves' examination being normal.
Motor system examination revealed symmetrically reduced bulk in all muscle groups, and muscles were tender to touch. The tone was decreased in all muscle groups.
- Power-upper limb: At shoulder joint 3/5, elbow joint 3/5, at wrist 2/5
- Lower limb: At hip joint, knee and ankle 3/5
- Neck muscles: Weak
- Reflexes-deep tendon reflexes were reduced. Sensory system and cerebellar functions were normal.
Other systems' examination was normal.
Routine investigations revealed elevated inflammatory markers such as erythrocyte sedimentation rate – 50 mm/1st h, lactate dehydrogenase – 1202 (230–440), C-reactive protein (CRP) – 6035 IU/L (26–140). Electromyography showed a myopathic pattern (short-duration, low-amplitude polyphasic on voluntary activation) and increased spontaneous activity with fibrillation suggestive of myopathic weakness. Thyroid function tests, serum parathyroid hormone levels were normal and viral markers such as HIV, Hepatitis B surface antigen, and Anti-HCV were negative. Malignancy markers such as carcinoembryonic antigen, CA-125, alpha-fetoprotein were within the normal limits. Antinuclear antibody and Anti-U1 RNP antibodies were negative. Hence, the possibility of mixed connective tissue disorder (MCTD) was ruled out. A normal cerebral spinal fluid and nerve conduction velocity ruled out acute inflammatory demyelinating polyneuropathy.
Following differential diagnosis was considered:
- Inflammatory myopathies
- MCTD – common in females with fatigue and myalgia being common presentation
- Myasthenia gravis occurs commonly in females; weakness of muscles of deglutition, the weakness can generalize and affect limb muscles as well
- Myopathy related to hypothyroidism-proximal muscle weakness in the lower limbs and fatigue
- Malignancy-Breast, lung, ovarian, and colorectal cancers are commonly associated with inflammatory myopathies.
After excluding all possible etiologies for myopathies, most likely etiology was inflammatory myopathy. A muscle biopsy was performed from quadriceps, and the reports thus obtained revealed a diagnosis of IBM. Subsequently, the patient was started on intravenous methyl prednisolone for 3–5 days, but she did not show any improvement. Subsequently, she was discharged on oral steroids and advised for rehabilitation.
| Discussion|| |
It is difficult to differentiate between the different types of inflammatory myopathies clinically.
The age-adjusted prevalence of IBM in people over the age of 50 is 3.5/100,000 making it the most common idiopathic inflammatory myopathy in this age group. DM is common in children and adult women while IBM is three times more frequent in men than women. Weakness and atrophy, especially foot extensors, occurs in almost all cases of IBM and may be a clue to early diagnosis. Some patients present with falls because of the collapse of knees due to early quadriceps weakness, others present with weakness in the small muscles of the hands, especially finger flexors, and complain of inability to hold objects such as a glass or perform tasks such as turning keys. Distal muscle involvement is late during the course of PM and DM but fairly early in the case of IBM. In case of DM, weakness is symmetrical and affects proximal muscles more than distal. Muscle atrophy and weakness are often asymmetrical and may lead to an erroneous diagnosis of motor neuron disease. However, unlike ALS, no significant atrophy of the hand intrinsics occurs, fasciculations are absent, and deep tendon reflexes are normal or reduced. Dysphagia is common, occurring in up to 60% of IBM patients and may lead to episodes of choking. Serum CK concentration should always be elevated in PM, unlike DM or IBM, in which the CK concentration may be normal. Sensory examination is normal. The pattern of distal weakness, which superficially resembles motor neuron or peripheral nerve disease, results from the myopathic process which affects distal muscles selectively.
IBM is both a myo-degenerative and neurodegenerative disease. The histopathological examination reveals rimmed vacuoles within the myofibril. The presence of inflammation alone can lead to an erroneous diagnosis of PM in cases where the inclusion bodies are not found. If there is no definite diagnosis, then a biopsy should be repeated. It is usually poorly responsive to steroids and another immunosuppressive (e.g., methotrexate) and immunomodulating (e.g., intravenous immunoglobulin) therapies. Occupational therapy may help a patient's ability to engage in activities of daily living. Patients with swallowing complaints should be referred to a speech therapist for proper education regarding consistency of diet and aspiration precautions. IBM has the least favorable prognosis of the inflammatory myopathies. Most patients will require the use of an assistive device such as a cane, walker, or wheel chair within 5–10 years of onset. In general, older age of onset in IBM is associated with the more rapidly progressive course.
| Conclusion|| |
IBM is still difficult to diagnose and unfortunately remains frequently misdiagnosed, with a delay in accurate diagnosis of just over 5 years, which has not changed over the last 25 years. IBM is rare and has no effective therapy thus; accurate diagnosis is of crucial importance in providing adequate patient counseling and information about the prognosis and course of the disease. It is important to keep in mind that it can present in the less common gender for this disease, i.e., females and should be suspected even in the younger age groups as has been highlighted by this case. Cases of suspected IBM should be referred to a center specializing in neuromuscular disease so that an appropriate diagnosis can be made and inappropriate treatments are avoided.
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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Fauci A, Hauser S, Jameson J, Kasper D, Longo D, Loscalzo J. Harrison's Principles of Internal Medicine. 19th
ed. New York, NY: McGraw-Hill Education LLC; 2015. p. 2194.
Brady S, Squier W, Hilton-Jones D. Clinical assessment determines the diagnosis of inclusion body myositis independently of pathological features. J Neurol Neurosurg Psychiatry 2013;84:1240-6.
Dimachkie MM, Barohn RJ. Inclusion body myositis. Semin Neurol 2012;32:237-45.
Dabby R, Lange DJ, Trojaborg W, Hays AP, Lovelace RE, Brannagan TH, et al.
Inclusion body myositis mimicking motor neuron disease. Arch Neurol 2001;58:1253-6.
Chilingaryan A, Rison RA, Beydoun SR. Misdiagnosis of inclusion body myositis: Two case reports and a retrospective chart review. J Med Case Rep 2015;9:169.
de Camargo LV, de Carvalho MS, Shinjo SK, de Oliveira ASB, Zanoteli E. Clinical, histological, and immunohistochemical findings in inclusion body myositis. Biomed Res Int 2018;2018:5069042.