Facial sensory loss in multi-bacillary leprosy patients.

Sensation over the face was estimated using the Semmes-Weinstein's monofilament (target force 0-05 gms) in a cohort of multi-bacillary (MB) patients whose clinical and ocular characteristics were available at the time of leprosy diagnosis. Among the 190 MB patients examined, 56 (30%) had areas of sensory impairment somewhere on the face and 43 (23%) had sensory deficit over the lids and/or the malar area. Lagophthalmos (adjusted OR 8.96, 95% CI 0.96-83.50), Type 1 reaction (aOR 2.47, 95% CI 1.11-5-52), history of reactions (aOR 6.36, 95% CI 2.40-16.85) and glove and stocking anaesthesia (aOR 3.49, 95% CI 1-40-8.70) were associated with impaired facial sensation. Hypoesthesia restricted to areas over the lids and/or malar area showed a stronger association with lagophthalmos (aOR 17.5, 95% CI 1.98-154.36). Loss of facial sensation appears to be associated with lagophthalmos in MB patients.

Manifestaçoes fonoaudiologicas em um grupo de doentes de Hanseniase / Manifestations fonoaudiologicas in a group of patients from leprosy

A Hansenise, doença cronica infecto-contagiosa e de nofificaçao compulsoria, e causada pelo bacilo de Hansen (Mycobacterium leprae), e pode apresentar multiplas lesoes em qualquer local do corpo...

Facial onset sensory and motor neuronopathy (FOSMN syndrome): a novel syndrome in neurology.

A 'syringomyelia-like' syndrome has been infrequently reported in neurological disorders such as Tangiers disease and lepromatous leprosy. This study reports a novel 'syringomyelia-like' syndrome in four adult male patients, which we have termed facial onset sensory and motor neuronopathy, or FOSMN syndrome, that appears to have a neurodegenerative aetiology. Clinical, neurophysiological and pathological data of four patients were reviewed, including the autopsy in one patient. Four male patients (mean age at onset 43), initially developed paraesthesiae and numbness in a trigeminal nerve distribution, which slowly progressed to involve the scalp, neck, upper trunk and upper limbs in sequential order. Motor manifestations, including cramps, fasciculations, dysphagia, dysarthria, muscle weakness and atrophy developed later in the course of the illness. Neurophysiological findings revealed a generalized sensory motor neuronopathy of caudally decreasing severity in all four patients. Autopsy in one patient disclosed loss of motoneurons in the hypoglossal nucleus and cervical anterior horns, along with loss of sensory neurons in the main trigeminal sensory nucleus and dorsal root ganglia. FOSMN syndrome appears to be a slowly progressive neurodegenerative disorder, whose pathogenesis remains to be determined.

Quantitative assessment of facial sensation in leprosy.

The trigeminal and great auricular nerves which supply sensation to the face are affected in leprosy. No objective sensory testing methods have been devised for testing sensation in the face. Testing for corneal sensation to ascertain trigeminal nerve or visualization and palpation of the great auricular nerve alone may not be enough to establish the involvement of these nerves. In a sample of leprosy patients, face sensation threshold measurements were done using a set of three Semmes-Weinstein (SW) monofilaments that gave a force of 0.05-0.07, 0.2 and 2 g. Sensation was tested by three examiners and intra- and inter-observer testing was used as a means to validate the findings. Within the limitations of this study, the results indicate that use of SW monofilaments is a fairly reliable and repeatable method for sensory testing in the face. During follow up, a single filament with a force of 0.5-0.7 g (2.83 marking number in SW filament or any other filament with a corresponding gram force) could be used to assess sensation. A simple procedure of quantifying sensation in these nerves is suggested. A method to incorporate trigeminal or great auricular nerve sensory testing into the existing sensory assessment charts is also discussed.