02582nas a2200313   4500000000100000008004100001260001200042653002400054653001200078653002600090653002700116100001600143700001300159700001300172700001400185700001400199700001100213700001400224700001500238700001700253700001200270700001500282700001200297700001400309700001400323245010400337520181300441022001402254       2021                            d  c10/202110aMycobacteria leprae10aleprosy10aperipheral neuropathy10aprimary neural leprosy1 aTomaselli P1 aSantos D1 aSantos A1 aAntunes D1 aMarques V1 aFoss N1 aMoreira C1 aNogueira P1 aNascimento O1 aNeder L1 aBarreira A1 aFrade M1 aGoulart I1 aMarques W00aPrimary neural leprosy: clinical, neurophysiological and pathological presentation and progression.3 a<p>Disability in leprosy is a direct consequence of damage to the peripheral nervous system which is usually worse in patients with no skin manifestations, an underdiagnosed subtype of leprosy known as primary neural leprosy. We evaluated clinical, neurophysiological and laboratory findings of 164 patients with definite and probable primary neural leprosy diagnoses. To better understand the disease progression and to improve primary neural leprosy clinical recognition we compared the characteristics of patients with short (≤ 12 months) and long (> 12 months) disease duration. Positive and negative symptoms mediated by small-fibre were frequent at presentation (∼95%), and symptoms tend to manifest first in the upper limbs (∼68%). There is a consistent phenotypic variability between the aforementioned groups. Deep sensory modalities were spared in patients evaluated within the first 12 months of the disease, and were only affected in patients with longer disease duration (∼12%). Deep tendon reflexes abnormalities were most frequent in patients with longer disease duration (p < 0,001), as well as motor deficits (p = 0,002). Damage to large fibres (sensory and motor) is a latter event in primary neural leprosy. Grade-2 disability and nerve thickening was also more frequent in cases with long disease duration (p < 0,001). Primary neural leprosy progress over time and there is a marked difference in clinical phenotype between patients with short and long disease duration. Patients assessed within the first 12 months of symptom onset had a non-length-dependent predominant small-fibre sensory neuropathy, whilst patients with chronic disease presented an asymmetrical all diameter sensory-motor neuropathy and patchily decreased/absent deep tendon reflexes.</p>  a1460-2156