Guillain-Barré syndrome-like presentation in borderline leprosy with type-2 reaction
Published Web Locationhttps://doi.org/10.5070/D31wj8g4tw
Guillain-Barré syndrome-like presentation in borderline leprosy with type-2 reaction1. Dept of Skin & STD, Kasturba Medical College, Manipal 576104, Karnataka. email@example.com
Dr Vandana Mehta Rai MD DNB1, Dr SD Shenoi MD1, and Dr SN Rao MD DM2
Dermatology Online Journal 12 (2): 21
2. Dept of Neurology, Kasturba Medical College, Manipal 576104, Karnataka
A 46-year-old man with borderline lepromatous leprosy with type-2 reaction being treated with multi-bacilliary-multiple drug therapy and steroids presented with an acute onset of flaccid quadriparesis. A nerve conduction study and CSF analysis were similar to that seen in Guillain Barre syndrome. Muscle weakness improved considerably with an increased dose of corticosteroid; after 6 months the patient recovered completely.
Guillain-Barré (GB) syndrome is an acute fulminant polyradiculopathy that is autoimmune in nature and characterized by an acute onset of muscle weakness that progresses more or less symmetrically over a period of 1-2 weeks. In about 75 percent of patients a mild respiratory or a gastrointestinal infection precedes the neurologic symptoms. The agents commonly implicated are Campylobacter jejuni, cytomegalovirus, Epstein Barr virus, human immunodeficiency virus, Mycoplasma pneumoniae, thrombolytic agents, lymphoma and vaccination. We report a case of 46-year-old man with borderline (BL) leprosy with type-2 reaction on treatment, who subsequently developed features of a Guillain-Barré-like syndrome.
A 46-year-old man from Davangere presented to the dermatology department with complaints of scrotal pain of 1-year duration, recurrent nodular lesions over upper and lower limbs of 8-months duration, and fever for 5 days. The skin lesions were tender and evanescent and generally subsided in 2-3 days. There were no features of neuritis, arthritis, myositis, or iritis. Testicular pain that had been present for the past year was being treated with scrotal support and anti-inflammatory drugs. Clinical examination revealed the presence of madarosis with multiple ill-defined, hypopigmented macules with intact sensation on the upper and lower limbs. Multiple tender nodules were present on the arms, ears and thighs. Greater auricular, ulnar, and lateral popliteal nerves were thickened bilaterally but were nontender; the patient exhibited loss of touch and temperature sensation bilaterally below the ankles. Motor examination was normal. The right testis and epididymis were tender to palpation. Laboratory investigation revealed the presence of anemia and an elevated ESR. Urinalysis showed the presence of protein with 8-10 RBCs/hpf and 2-4WBCs/hpf. Slit skin smear showed bacillary index of 2+. A scrotal Doppler examination showed right sided epididymorchitis. The patient was diagnosed with borderline lepromatous leprosy with type-2 reaction and he was discharged with multi-bacilliary-multiple drug therapy (MB-MDT, rifampin, Dapsone, and clofazimine) and prednisolone 30 mg daily to be gradually tapered by 5 mg/week. The usual clofazimine dose was increased to 200 mg/day.
The patient presented 3 ½ months later with weakness of proximal muscles of the upper and lower extremities. This weakness had an insidious onset and was progressed gradually, combined with tingling and pain in the legs. At this time he was on 10 mg prednisolone along with (MB-MDT). Although his reactional status was not severe he had difficulty in walking and getting up from a squatting position and lifting a hand above shoulder level. There was no history of bladder dysfunction. On further questioning he gave a history of fever and diarrhea 3 weeks earlier that had lasted for 2 days. A neurological assessment revealed higher mental functions and cranial nerves to be normal. The muscle bulk was normal but tone was decreased bilaterally. The patient's hand grip was weak bilaterally with grade-2 weakness of the elbow extensors and grade-4 weakness of the shoulder, elbow flexors and wrist muscles. Dorsiflexion and plantarflexion of toes was weak bilaterally with grade-4 weakness at left hip and ankle and grade-3 weakness of right hip and bilateral knee joint muscles. The deep tendon reflexes were absent bilaterally. In view of the proximal muscle weakness and areflexia a clinical diagnosis of Guillain-Barré syndrome was considered. Nerve conduction velocity study revealed a mild increase in motor latency with a decrease in the amplitude in the motor nerves of upper and lower limbs. The CSF examination showed a marginal rise in protein count with no cells. The dose of steroids was increased to 60 mg and within 2 weeks the muscle weakness improved considerably and the patient was able to walk without support. At a 6 month follow up examination, although there was mild tenderness in the scrotum with occasional ENL the muscle strength had returned to normal.
GB syndrome is an acquired disease of the peripheral nerves that is characterized clinically by rapidly progressing paralysis, areflexia, and albumino-cytological dissociation in cerebrospinal fluid. It is autoimmune in nature and affects both sexes; in the post polio era it is the most common cause of an acute generalized paralysis . A prior respiratory or gastrointestinal infection with Campylobacter jejuni followed by an ascending paralysis that evolves over hours to a few days is documented in 75 percent of patients. The legs are more frequently affected than the arms. The lower cranial nerves may also be involved and 30 percent of patients may require ventilatory assistance. Deep tendon reflexes usually disappear within first few days of onset. Bladder dysfunction may occur in severe cases but is usually transient. GB syndrome has also been reported after influenza vaccination . Several types have been reported in the literature, each with distinctive electrodiagnostic and pathologic features. Our patient had features of the acute inflammatory demyelinating type (AIDP). Circumstantial evidence suggests that GB syndrome is a result of immune responses to agents such as infections or vaccines that damage host nerve tissue attributed to resemblance of epitopes (molecular-mimicry mechanism). The neural targets are gangliosides (basic protein P2 found in the peripheral nerves) that are typically exposed on the plasma membrane of cells, rendering them susceptible to an antibody-mediated attack. Only one case has been reported in the literature, highlighting the occurrence of a severe GB syndrome in a patient with lepromatous leprosy with type-2 reaction . Patients with lepromatous leprosy who develop ENL have higher levels of tumor necrosis factor alpha (TNF-α) as compared to those who do not develop ENL  and TNF-α is regarded as one of the immune factors that can induce demyelination of peripheral nerves in patients with GB syndrome . Although a typical case of GB syndrome presents with an acute onset of flaccid quadriparesis, multiple cranial nerve palsies, severe respiratory muscle weakness and albumino-cytological dissociation in cerebrospinal fluid our patient presented with features of type-2 reaction (fever, ENL, epididymorchitis), acute onset muscle weakness, areflexia and a marginal rise in the protein count in cerebrospinal fluid. The cause of our patient's GB-like presentation could be attributed to cell injury caused by type-2 reaction that exposed the neural antigens and incited an autoimmune reaction . Since his reactional status was not severe and he had a prior history of diarrhea and fever, the cause of the GB-like occurrence could not be absolutely attributed to either. Our patient was treated with high dose corticosteroids along with the MDT that produced a complete recovery of his muscle weakness.
References1. Vedanarayan VV, Chaudhry V. Guillain Barre Syndrome recent advances. Indian J. Pediatr 2000;67: 635-46
2. Schonberger LB, Bregman DJ, Sullivan Bolyai JZ, Keenlyside RA, Ziegler DW, Retailliau HF. Guillain Barre Syndrome following vaccination in the National Influenza Immunization Program, United States, 1976-1977. Am J .Epidemiol 1979;110:105-23
3. Grover C, Kubba S, Nanda S, Kumar V, Reddy BS. Leprosy with Guillain Barre Syndrome: a neurologic manifestation? J Dermatol 2004;31:119-23
4. Partida Sanchez S, Favila Castillo L, Pedraza Sanchez G. IgG antibody subclass tumor necrosis factor and IFN-δ levels in patients with Type 2 reaction on thalidomide treatment. Int Arch Allergy Immunol 1998;116:60-6
5. Radhakrishnan VV, Suni MG, Reuber S, Mathai A. Circulating tumor necrosis factor alpha and soluble TNF receptors in patients with Guillain Barre Syndrome. Indian J Med Res 2003;117:216-20
© 2006 Dermatology Online Journal