A unique case of concomitant intra and extracranial Hansen's disease.

Leprosy is a debilitating disease that usually involves the peripheral branches of the cranial nerves leading to anesthetic/hypoesthetic skin lesions and thickened peripheral nerves. However, the involvement of the central nervous system (CNS) is extremely rare. To the best of the author's knowledge, the involvement of the cranial nerve nuclei by leprosy has not been reported in the literature and the present case is the first report of involvement of the facial nerve nuclei by leprosy.

Cranial nerve involvement in patients with leprous neuropathy.

BACKGROUND: Leprosy is one of the most common causes of peripheral neuropathy, perhaps closely matched by diabetic neuropathy. Patterns of peripheral neuropathy in leprosy can be varied, which may include mononeuropathy, mononeuritis multiplex and symmetric polyneuropathy. Cranial nerves, especially facial and trigeminal nerves, are also commonly involved in leprosy. AIMS: To find out the pattern and spectrum of cranial nerve involvement in a consecutive series of patients with leprous neuropathy. SETTINGS AND DESIGN: A retrospective review of patients admitted with leprosy to the Neurology Department of a tertiary care center. MATERIALS AND METHODS: All consecutive patients admitted during an 8-year period (1995-2003) and diagnosed to have leprosy were included. They were clinically evaluated to determine the frequency and pattern of cranial nerve involvement. RESULTS: About 18% (9/51) of the leprosy patients seen during that period had clinical evidence of cranial nerve involvement. Facial and trigeminal nerves were the most commonly affected (five and four patients respectively). CONCLUSIONS: Cranial nerve involvement is common in leprosy, which emphasizes the need to carefully examine them. Also, one should exclude leprosy in patients presenting with isolated cranial neuropathies.

Leprosy affects facial nerves in a scattered distribution from the main trunk to all peripheral branches and neurolysis improves muscle function of the face.

Current literature rejects nerve release in leprous facial neuropathy and states that lesions are restricted to the peripheral zygomatic branches. Since there are approximately 500,000 patients with this disease throughout the world, we wanted to clarify the precise location of facial nerve's affection and the benefit of neurolysis. Our study showed that in patients with leprosy, the facial nerve's main trunk, the peripheral zygomatic branches, and all other branches were affected. Follow-up showed improvement in lagophthalmos and in misreinnervation, with no improvement in the control cohort. Nerve release improves muscle function in leprous facial neuropathy, provided surgery is performed on all affected segments. Intraoperative electroneurodiagnostics is an effective tool for detecting the most proximal site of lesion and ensuring effective surgery.

Leprosy affects facial nerves in a scattered distribution from the main trunk to all peripheral branches and neurolysis improves muscle function of the face

Current literature rejects nerve release in leprous facial neuropathy and states that lesions are restricted to the peripheral zygomatic branches. Since there are approximately 500,000 patients with this disease throughout the world, we wanted to clarify the precise location of facial nerve's affection and the benefit of neurolysis. Our study showed that in patients with leprosy, the facial nerve's main trunk, the peripheral zygomatic branches, and all other branches were affected. Follow-up showed improvement in lagophthalmos and in misreinnervation, with no improvement in the control cohort. Nerve release improves muscle function in leprous facial neuropathy, provided surgery is performed on all affected segments. Intraoperative electroneurodiagnostics is an effective tool for detecting the most proximal site of lesion and ensuring effective surgery.

Facial nerve pathology in leprosy: searching for the proximal extent of the lesion in facial nerve biopsies.

A light and electron microscope study was made of resin embedded facial nerves in three cases of leprosy involving the facial nerve. The patients had irreversible facial nerve palsies and had requested facial reconstruction. No consistent pattern of nerve fibre damage was found. In one case the temporozygomatic was affected, but the cervical branch was normal, suggesting the damage begins distally. In two cases the loss of nerve fibres in the trunk and all branches was similar, and is likely to emanate from damage at a more proximal site. The presence of increased numbers of unmyelinated axons, often in clusters, is evidence of regeneration. These axons probably have the potential to develop into functional myelinated fibres provided that they can innervate a viable distal target such as a muscle graft. These regenerating axons are distal to the stylomastoid foramen suggesting that the most proximal level of involvement of the facial nerve could be intracranial. The finding of a more proximal level of nerve involvement, implies that the mis-reinnervation seen in partially recovered facial nerve palsies in leprosy, could be due to some regenerating axons being mis-directed at the level of the main trunk bifurcation.

[Immunohistochemical study on temporal bone of autopsy cases with Mycobacterium leprae infection].

Three temporal bones were obtained en bloc from autopsy cases with lepromatous leprosy from the middle cranial fossa side after removing the brain. After fixation with 10% formalin followed by sufficient decalcification, the specimens were embedded in paraffin en bloc and cut serially to stain every 10th section with hematoxylin and eosin (H&E) for anatomical orientation. An immunohistochemical study with anti-neurofilament, anti-MBP (myelin basic protein), anti-BCG (Bacillus Calmette-Gue'rin) and anti-PGL (Mycobacterium leprae-specific antiphenolic glycolipid-I) antibodies were performed to vestibular, cochlear and facial nerves, respectively, on the basis of anatomical orientation of the adjacent H&E sections. In one of three cases, positive staining by anti-PGL antibodies was recognized only in the facial nerve both in its internal auditory meatal and tympanic portions. However, even in this case, no neural damage was observed either by anti-neurofilament nor anti-MBP stainings. This finding supports the possibility of central neural infection by Mycobacterium leprae.

Reaçäo hansênica tipo I na hanseníase borderline tuberculóide durante a poliquimioterapia: época de aparecimento e nervos acometidos / Type I reaction in borderline tuberculoid leprosy under multidrug therapy: time of onset and nerves affected

FUNDAMENTOS - A reaçäo tipo I é pouco estudada na literatura. OBJETIVOS - Descrever a época de aparecimento da reaçäo tipo 1 e os nervos acometidos, nos pacientes portadores de hanseníase borderline tuberculóide (BT), durante a poliquimioterapia(PQT). PACIENTES E MÉTODOS - Estudo longitudinal realizado no Ambulatório de Dermatologia da Faculdade de Medicina da UFMG. Setenta e um pacientes com hanseníase BT foram incluídos no estudo de agosto de 1989 a agosto de 1993. RESULTADOS - 89,3 por cento dos pacientes hansenianos BT com reaçäo tipo 1 apresentaram esse surto atá a sexta dose de tratamento. O nervo ulnar foi o mais acometido nas reaçöes tipo 1 (37,0 por cento). CONCLUSÃO - A reaçäo tipo 1 nos pacientes hansenianos BT ocorre mais frequentemente até a sexta dose de PQT. O nervo ulnar foi o mais acometido

Pathology and pathogenesis of predilective sites of nerve damage in leprous neuritis. Nerves in the arm and the face.

A very brief review of the literature on the clinicopathological aspects of leprosy is given; mainly through references. The salient features of the two main types of leprosy--tuberculoid and lepromatous--are presented in a Table. The surgical and pathological findings are briefly described and the pathogenesis of involvement of the facial nerve (a motor nerve) is discussed. On the basis of (i) the severe histopathologic changes (degeneration) of the most distal part of the zygomatic branch of the facial nerve (innervating the orbicularis oculi), with sparing of the roots of all branches and the trunk of the nerve; (ii) the concurrent loss of cutaneous sensations in the territory of the maxillary division of the trigeminal nerve (the region of the zygoma and the lower eyelid); and (iii) the close approximation or even anastomoses occurring between the ultimate branches of these two nerves, it is postulated that paralysis of orbicularis oculi occurs secondarily to the sensory nerve damage, i.e. due to spread of infection from the sensory branches of the trigeminal to the motor branches of the facial nerve. The surgical and pathological findings of the nerves in the arm, especially the ulnar and the median and their branches are described, in tuberculoid and lepromatous leprosy. The forms of nerve degeneration, the occasional axon regeneration, and the role of the Schwann cell in harbouring the bacilli, are illustrated. The lysosomal enzyme activity in the Schwann cells of nerve fibres, particularly of unmyelinated fibres which preferentially phagocytose the M. leprae, and their role, albeit not very successful, in degrading the bacilli and controlling the infection, are also stressed, through light and electronmicrographs. The constellation of secondary factors of the terrain operating to produce further damage to primarily diseased nerves, is discussed. These factors include indirect compression from unyielding fibroosseous tissues, minor traumata, stretching of the nerves, and their exposure to lower temperatures in subcutaneous tissues.

Lesions of the facial and trigeminal nerve in leprosy. An evaluation of 43 cases.

Facial and trigeminal nerve involvement was studied in 43 leprosy patients, 5.7% of the 750 Thai national in-patients of a leprosy rehabilitation centre. Involvement of the facial nerve occurred late in the disease and had an average duration of 12.1 years. The zygomatic branches of this nerve were the most frequently affected. Hypaesthesia and anaesthesia were most often observed in the maxillary divisions. Periorbital surgical procedures to prevent severe ocular complications are evaluated and discussed.

Prevalence of leprosy among slum dwellers.

Distribution of leprosy among slum dwellers in two areas of Bangalore City were studied. Out of a random sample of 483 it was found that 150 were suffering from leprosy while 74 tuberculoid and 25 lepromatous cases were detected, the remaining showed up as borderline and polyneuritic types. The important socio economic features and the consequences of this chronic disease have been discussed.