|Year : 2019 | Volume
| Issue : 2 | Page : 136-138
Syringomyelia diagnosed with leprosy: A case report
Vijay Sardana, Rahi Kiran Bhattiprolu
Departments of Neurology, Government Medical College, Kota, Rajasthan, India
|Date of Web Publication||15-Jul-2019|
Rahi Kiran Bhattiprolu
Government Medical College, Kota, Rajasthan
Source of Support: None, Conflict of Interest: None
Leprosy has high incidence in tropical and subtropical countries. We document a case of 26-year-old female with an 18-month history of insidious onset progressive weakness and wasting of small muscles of both hands with decreased sensation distal to elbows and trophic changes without thickened nerves. The patient was on anti-leprosy treatment for the past 1 year with no response to treatment. Examination revealed diminished reflexes in the upper limbs and preserved reflexes in the lower limbs. Skin biopsy was normal. Magnetic resonance imaging of the spine was suggestive of Arnold–Chiari malformation type I with large cervico-dorsal syrinx which was ultimately treated by the surgical management. We suggest that all cases of leprosy should include syringomyelia as a differential diagnosis so that unnecessary prolonged exposure to anti-leprosy drugs with potential side effects can be prevented.
Keywords: Arnold-Chiari malformation type I, leprosy, syringomyelia
|How to cite this article:|
Sardana V, Bhattiprolu RK. Syringomyelia diagnosed with leprosy: A case report. Clin Dermatol Rev 2019;3:136-8
| Introduction|| |
Leprosy is one of the oldest diseases known with high incidence in tropical and subtropical countries. Mycobacterium leprae was the first bacterium to be identified as causing disease in humans. Neurological manifestations of leprosy have a significant overlap with syringomyelia. Leprosy affects pain and temperature sensation and may cause syringomyelia-like syndrome. Diagnosis is occasionally very difficult and some cases were later diagnosed with syringomyelia. In this report, we discuss a 26-year-old female being treated as a case of leprosy later diagnosed with Arnold-Chiari malformation type I with syrinx formation.
| Case Report|| |
A 26-year-old female presented with an 18-month history of insidious onset progressive weakness and wasting of small muscles of both hands [Figure 1] with decreased sensation distal to elbows and trophic changes with a 2-month history of progressive bulbar symptoms without the bladder and bowel involvement. On examination, there is diminished biceps and supinator jerks, preserved triceps, knee and ankle reflexes with extensor plantar bilaterally, and no thickened nerves. The patient was on anti-leprosy treatment for the past 1 year started empirically elsewhere (no documents) with no response to treatment. Nerve conduction study was suggestive of axonal neuropathy involving upper limbs. Skin biopsy taken from hypoesthetic area on the left forearm was not suggestive of Hansen's disease. Magnetic resonance imaging (MRI) of the brain was normal. MRI cervical spine showed herniation of cerebellar tonsil with large cervico-dorsal syrinx formation and features suggestive of Arnold-Chiari malformation Type I [Figure 2].
|Figure 1: Patient with wasting of small muscles of the hand and finger deformities|
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|Figure 2: Sagittal and axial section of T1-weighted and T2-weighted magnetic resonance imaging cervical spine showing herniation of cerebellar tonsil with large cervico-dorsal syrinx|
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| Discussion|| |
With the introduction of multi-drug therapy, occurrence of new leprosy cases has reduced to a large extent, but efforts to eliminate leprosy were only partially successful. In India, the prevalence of leprosy reduced from 58/10,000 population in 1980 to 0.69/10,000 in 2010, thus eliminating leprosy as a public health problem. Still, new cases are diagnosed because of continued disease transmission. Leprosy diagnosis depends on the demonstration of one or more of the three cardinal signs – anesthetic/hypoesthetic skin patches, thickened peripheral nerves, and acid-fast bacilli in skin smear. Delayed treatment of leprous neuropathy is the major cause of disability and social isolation in these patients, which might be the reason for starting empirical therapy in our patient. The diagnosis of neural leprosy is occasionally very difficult due to its close resemblance to many other diseases. Among these, the occasional resemblance to syringomyelia is of interest as both will have long history of sensory motor quadriparesis with trophic changes. Usually, clinical manifestations in leprosy involve lower limbs more than upper, whereas in syringomyelia, lower limbs may not be involved early in the disease course like in our case. In leprosy, pathology starts in the skin and extends along the nerves in gradually diminishing degree from the periphery toward the spinal cord. It is pronounced in exposed areas such as that of the ulnar nerve at the elbow and the peroneal nerve behind the head of the fibula. Syringomyelia is a complexus of symptoms dependent on lesions of the central spinal cord resulting in structural alterations that terminate in the formation of cavities. It is usually caused by developmental defects, trauma, and inflammatory pathology. Cases of syringomyelia rarely include leprosy in the differential diagnosis, but almost all cases of leprosy refer to syringomyelia as the patient will have sensory impairment along with preserved reflexes till late stages. Although both have chronic course, the presence of constitutional symptoms, glove and stocking sensory loss, and patchy sensory loss favors leprosy. Muscular atrophy in leprosy corresponds in distribution with the cutaneous sensory loss while its not the case in syrinx where dissociative anesthesia in segmental distribution is seen. Our case was already on anti-leprosy treatment since past 1 year without any response with recent-onset bulbar symptoms which are associated with syringomyelia. In leprosy, bulbar symptoms are rare, trigeminal, and facial nerves that are commonly involved. However, in syringomyelia, facial involvement is rarely seen. There were diminished reflexes in the upper limbs and brisk reflexes in the lower limbs in our patient, which is usually seen in syringomyelia. Leprosy can have similar presentation manifested as pseudo syringomyelitic form due to involvement of dorsal root ganglia. MRI scan in our patient showed cerebellar tonsillar herniation with large cervico-dorsal syrinx features consistent with Arnold-Chiari malformation type I. In case of spinal cord involvement in leprosy, degeneration of posterior columns and amyloid bodies in gray and white matter is seen. According to the National Institute of Neurological Disorders and Stroke, treatment of syringomyelia is usually surgical, especially in the associated presence of Chiari malformation. Our patient was operated successfully and is now under regular follow-up.
| Conclusion|| |
In India, due to high incidence of leprosy and its varied clinical presentations, empirical treatment is usually started in many centers. However, all cases of leprosy should include syringomyelia as a differential diagnosis due to resembling clinical features. The drug treatment of leprosy is long and may expose the patient to unnecessary side effects if given unnecessarily.
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.
The authors would like to thank the entire Department of Neurology, Government Medical College Kota who had made the preparation of this case report possible.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]