|Year : 2006 | Volume
| Issue : 3 | Page : 166-168
Acute disseminated encephalomyelitis in Hansen's disease
J Jacob1, M Alexander1, S Aaron1, S Pulimood2, N Walter3, C Gnanamuthu3
1 Department of Neurological Sciences, Christian Medical College Hospital, Vellore, India
2 Department of Dermatology, Christian Medical College Hospital, Vellore, India
3 Department of Pathology, Christian Medical College Hospital, Vellore, India
Department of Neurological Sciences, Christian Medical College Hospital, Vellore - 632 004
Source of Support: None, Conflict of Interest: None
The authors report a 24-year-old man with Hansen's disease in Type 1 reaction, developing a demyelinating disorder affecting the central and peripheral nervous system. He responded well to plasma exchange and intravenous methyl prednisolone. The neural damage could have been due to cross-reacting epitopes causing molecular mimicry and resultant demyelinating disorder.
Keywords: Demyelination, Hansen′s disease, lepra reaction
|How to cite this article:|
Jacob J, Alexander M, Aaron S, Pulimood S, Walter N, Gnanamuthu C. Acute disseminated encephalomyelitis in Hansen's disease. Ann Indian Acad Neurol 2006;9:166-8
|How to cite this URL:|
Jacob J, Alexander M, Aaron S, Pulimood S, Walter N, Gnanamuthu C. Acute disseminated encephalomyelitis in Hansen's disease. Ann Indian Acad Neurol [serial online] 2006 [cited 2020 Nov 27];9:166-8. Available from: https://www.annalsofian.org/text.asp?2006/9/3/166/27660
| Introduction|| |
Hansen's disease is caused by Mycobacterium leprae , a slow growing acid-fast bacillus. The incubation period is very long and the disease manifestation depends on the status of the host T cell immunity. Although T cell immunity by itself is protective, a person who contracts infection and if the T cell immunity is turned on, bacillary growth is restricted but the resultant nerve damage is profound as in Tuberculoid leprosy. When the host T cell immunity is very low, there will be widespread dissemination resulting in lepromatous leprosy and then, there is the borderline disease which forms the spectrum between the two immunological subtypes of the disorder.
Type 1 reaction/reversal reaction occurs with abrupt increase in DTH against M. leprae antigenic determinants released from Schwann cell with resultant nerve damage.
In the following report, the authors document acute disseminated encephalomyelitis (ADEM) in a patient with Hansen's disease in Type 1 reaction.
| Case Report|| |
This 24-year-old patient was apparently well until 6 months before when he developed weakness and decreased sensation of the 4th and 5th digits of both hands and had hypo pigmented anesthetic patches over his trunk. He was diagnosed to have Hansen's disease and was started on clofazimine and had received two injections of BCG. He continued the medications until he developed pain in the distribution of the right ulnar nerve. He was treated with a course of steroids and then underwent decompression surgery for the nerve. About a month later, he developed subacute onset quadriparesis, involving both the proximal and distal muscles with no history of bladder dysfunction. There was no history of fever, arthralgia/arthritis or subcutaneous swellings. Examination revealed about 15 cutaneous patches ranging in size from 4 to 20 cm across. The skin lesions were present on the trunk, gluteal region and limbs and were well defined, icthyotic, erythematous, edematous and hypo-esthetic. Neurological examination revealed no cranial nerve palsies, flaccid tone of all the four limbs with no wasting. The motor power was 2/5 MRC grade in the proximal and intermediate muscles of the upper and lower limbs and grade 0 in the distal muscles. The deep tendon reflexes were absent in the upper limbs, the knee jerks' brisk and the ankle jerks were absent. Sensory examination revealed sensory loss from C5 to T1 dermatomes. In view of the dropped upper limb reflexes and segmental sensory loss, an MRI of the cervical spine was done which showed T2 weighted hyper intensity from C4 to C7. The lesion was expansile and showed patchy enhancement [Figure - 1].
Detailed nerve conduction studies revealed no motor or sensory evoked response from all the four limbs. CSF examination showed 10 WBCs (all lymphocytes), 98 mg/dl of sugar and 49 mg/dl of protein. Skin biopsy [Figure - 2] from the lesion over the left arm showed clusters of lymphocytes and histiocytes, the latter forming ill- defined granulomas in association with a few Langhans giant cells. Occasional granulomas were present in dermal nerves and acid-fast bacilli were not found. The skin biopsy was diagnostic of Tuberculoid type Hansen's disease. This patient was in Type 1 lepra reaction, with features of both central and peripheral nervous system demyelination. He was treated with three plasma exchange sessions, during, which 4 l of plasma were removed and a week later was given Intravenous Methyl prednisolone 1G/day x 3 due to the lack of clinical improvement. He was started on clofazamine 50 mg daily and dapsone 100 mg daily. At discharge, he was started on oral Prednisolone 80 mg on alternate days for 1 months, which was to be tapered and stopped over 3 months and azathioprine 100 mg daily. On follow up visit, about a month later, the motor power had improved to grade 3/5 in the upper limbs, 4/5 in the proximal and intermediate groups of lower limb muscles with a distal power of 2/5 at the ankle and was ambulant with mild support. When reviewed after six months, he could walk unaided and the motor power in the upper and lower limbs was MRC grade 4/5. Follow up nerve conduction study showed normal DL, prolonged F latencies, mildly reduced CMAPs', mild reduction of motor conduction velocities of Median, Ulnar and Tibial nerves and absent sensory nerve action potentials.
| Discussion|| |
This patient with Hansen's disease had developed a reversal reaction within 6 months from the date of diagnosis. While on treatment with clofazimine and following two doses of BCG vaccine, he developed a reversal reaction and about a month later developed sub acute onset quadriparesis with absent deep tendon reflexes in upper limbs, brisk knee jerks and absent ankle jerks, segmental sensory loss from C5 to T1 bilaterally and no bladder dysfunction. MRI cervical spine showed evidence of involvement of the cervical cord from C4 to C7, which was in keeping with a demyelinating disorder. The nerve conduction study showed evidence of a severe poly neuropathy, as evidenced by absent motor and sensory evoked responses in all four limbs, which is usually not seen in Tuberculoid or Lepromatous neuropathy. The skin biopsy was typical of Tuberculoid leprosy and the patient was in Type 1 reaction, with features of central and peripheral nervous system demyelination. In view of the involvement of the cervical cord and the peripheral nervous system, a diagnosis of ADEM was made and treated with Plasma Exchange followed by Methyl Prednisolone. On analyzing the nerve conduction studies, initial study showing absence of motor and sensory evoked responses followed by the follow up study done 6 months later, when F latencies were prolonged with mild reduction of CMAPs' and mildly reduced motor conduction velocities indicate a recovering polyradiculo-neuropathy, most probably demyelinating. ADEM is an uncommon inflammatory demyelinating disease of the central nervous system that occurs usually following a systemic viral infection or following an immunization or vaccination., Most of the antecedent infections are viral, however, there have been reports of postmalarial neurological syndrome and ataxia, which was hypothesized as a consequence of ADEM. The prevailing hypothesis that post infectious encephalomyelitis results from a transient autoimmune response towards myelin or other self- antigens, possibly by molecular mimicry or by non- specific activation of non specific auto reactive T cell clones. T cells directed to microbial epitopes, may recognize amino acid sequences shared with myelin antigens, become activated and cross the endothelium of the BBB and attack the CNS myelin by molecular mimicry or along with antibodies.,
Immunotherapy with BCG vaccine is given as an adjunct to chemotherapy to enhance bacterial killing as well as bacterial clearance, does not reduce or may even increase the incidence of reversal reaction. Type 1 reaction/reversal reaction occurs with abrupt increase in DTH against M. leprae antigenic determinants released from the Schwann cells with resultant nerve damage. The possible hypotheses for development of ADEM in this patient could be these:
The BCG vaccine administered, was an antigenic challenge and recognized by the T cell, which were already primed to the Mycobacterium leprae. These T cells would have recognized homologous epitopes shared with the host myelin, got activated and attacked the CNS myelin by molecular mimicry, along with antibodies.
The other possibility is that bacterial super antigens from the mycobacterium could have triggered auto reactive T cells and this could have led to CNS damage.
Given the clinical setting, imaging and excellent response to immunotherapy, this patient had a monophasic demyelinating disorder (ADEM).
The relevance of this paper is that immune mediated demyelination of the Central Nervous system (ADEM) has not hitherto been reported in Hansen's disease.
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[Figure - 1], [Figure - 2]
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