Epithelioid malignant peripheral nerve sheath tumour of the ulnar nerve around the elbow: a diagnostic and therapeutic challenge.

Epithelioid malignant peripheral nerve sheath tumour (EMPNST) is a rare histological subtype of malignant peripheral nerve sheath tumour (MPNST), accounting for 5% to 17% of MPNSTs. The clinical and MRI findings of EMPNST mimic those of nerve abscesses, similar to the presentation in Hansen's disease. We present one such case with this kind of diagnostic dilemma. Intraoperative findings suggest a tumour changed the course of management subsequently. The development of neurological deficits postoperatively after tumour resection was a reconstructive challenge. To provide motor power and sensation through a procedure that provides a complete functional outcome for a young patient, distal nerve transfers were chosen. This provided an improvement in the quality of life and hastened the neurological recovery of the involved limb. Level of evidence: V.

Neuritic Leprosy; An Intriguing Re-visit to a Forbidden Ailment.

Leprosy is a chronic infectious disease that presents with varied manifestations. Pure neuritic leprosy is one of the rarest forms of the disease which is characterized by nerve involvement without the characteristic cutaneous stigmata. Eleven year old, healthy male presented with progressively increasing painful swelling at the medial aspect of the arm near to the right elbow joint with difficulty in extending right ring and little fingers at interphalangeal joint and numbness in the same region for last 1 year with no cutaneous abnormalities. Physical examination revealed 6x3 cm firm, tender lesion 3 cm proximal to the right elbow joint with positive tinel's sign, without signs of inflammation, along with characteristic claw hand deformity of right hand and atrophy of hypothenar and interossei muscle. Electro-diagnostic testing revealed findings consistent with a right ulnar axonal neuropathy above the elbow. Magnetic resonance imaging revealed well defined heterogeneously hyper intense linear lesion along the course of thickened ulnar nerve in the distal arm extending posterior to the medial condyle. It also showed an oval shaped lesion (2.1x1.0 cm) arising from the same segment of the nerve, without any bony or muscular involvement of that area. The patient underwent surgical exploration and ulnar nerve decompression with biopsy. Pathology revealed necrotizing granulomatous inflammatory acid fast bacilli stain negative lesion, which was histologically consistent with caseous abscess caused by tuberculoid leprosy, pathognomonic for Hansen's disease. He has been started on antibiotic therapy and is referred to leprosy center for further course of management. Pure neuritic leprosy, though rare, should be considered as differential diagnosis in cases presenting with peripheral neuropathy at leprosy-endemic areas. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Histoid leprosy: a retrospective clinicopathological study from central Nepal.

BACKGROUND: Histoid leprosy is a rare variant of lepromatous leprosy characterized by varied morphological and histopathological appearance while having a high bacillary load. These factors contribute to an ominous threat to the elimination status of leprosy, whereby these patients may act as a reservoir of infection. OBJECTIVE: To identify the clinicopathological characteristics of histoid leprosy in Chitwan, Nepal. METHODS: A retrospective hospital-based study spanning a period of 6 years was carried out at our department. All cases clinically and histopathologically suggestive of histoid leprosy were included in our study, and all relevant data were recorded and analyzed as per standard protocol. RESULTS: Histoid leprosy comprised 2.9% of all leprosy cases. Mean age of 39.45 years and male:female ratio of 1.75:1 were seen. Previous history of leprosy was obtained in 72.7%, and de novo development of histoid leprosy took place in 27.3%. Papules were the most common lesion seen, and upper limbs were the most frequent site of involvement, and the ulnar nerve was enlarged in most cases. Mean bacillary index was 5.39. Histopathology showed epidermal atrophy, positive Fite-Faraco stain for lepra bacilli, spindle-shaped histiocytes arranged in various patterns, and a well-circumscribed area of cells in the dermis in all cases. Grenz zone and pseudocapsule were seen in the majority of patients. All cases responded well to multibacillary multidrug therapy (MB-MDT) of 2 years. CONCLUSION: A high index of suspicion is essential for diagnosing histoid leprosy, both clinically and histopathologically.

High resolution sonographic examination: a newer technique to study ulnar nerve neuropathy in leprosy.

Objectives: A prospective case control study was conducted to calculate normal dimensions of ulnar nerve and study the size, echotexture and morphologic alterations in ulnar nerve in patients with leprosy. Methods: The study group included 48 patients diagnosed with leprosy on basis of clinical, bacteriologic and/or histopathologic evaluation. Sonographic measurements were taken at 3 levels around elbow. The morphology and vascularity were also studied. Control group consisted of 60 clinically normal ulnar nerves, on which ultrasound was performed to calculate normal parameters. 96 Ulnar nerves were independently evaluated clinically and on ultrasound. The mean cross sectional area and diameters (both antero-posterior and mediolateral) of controls at all three levels were calculated. The normal sonographic dimensions of ulnar nerve were calculated based on Mean ± 2SE and beyond the upper limit of normal was considered enlarged on ultrasound. Statistical analysis was done using SSPS version 17.0. Results: The dimensions of ulnar nerve were significantly larger in leprosy group for all levels (P value , 0.001). Sonographic abnormalities included hypoechoic areas (61·45%), loss of fascicular pattern (same 61·45%) and focal hyperechoic areas (48·95%). 37·5% of nerves (6 out of 16) with clinical evidence of reaction showed endoneural vascularity. Conclusions: We conclude that by detecting enlargement and/or morphologic alterations of ulnar nerve, sonography can objectively determine involvement of ulnar neuropathy in leprosy.

Ulnar nerve sonography in leprosy neuropathy.

A 23-year-old woman presented with a half-year history of right forearm sensory and motor dysfunction. Ultrasound imaging revealed definite thickening of the right ulnar nerve trunk and inner epineurium, along with heterogeneous hypoechogenicity and unclear nerve fiber bundle. Color Doppler exhibited a rich blood supply, which was clearly different from the normal ulnar nerve presentation with a scarce blood supply. The patient subsequently underwent needle aspiration of the right ulnar nerve, and histopathological examination confirmed that granulomatous nodules had formed with a large number of infiltrating lymphocytes and a plurality of epithelioid cells in the fibrous connective tissues, with visible atypical foam cells and proliferous vascularization, consistent with leprosy. Our report will familiarize readers with the characteristic sonographic features of the ulnar nerve in leprosy, particularly because of the decreasing incidence of leprosy in recent years.

Asymmetric Nerve Enlargement: A Characteristic of Leprosy Neuropathy Demonstrated by Ultrasonography.

BACKGROUND: Neurological involvement occurs throughout the leprosy clinical spectrum and is responsible for the most feared consequences of the disease. Ultrasonography (US) provides objective measurements of nerve thickening and asymmetry. We examined leprosy patients before beginning multi-drug therapy aiming to describe differences in US measurements between classification groups and between patients with and without reactions. METHODOLOGY/PRINCIPAL FINDINGS: Eleven paucibacillary (PB) and 85 multibacillary (MB) patients underwent nerve US. Twenty-seven patients had leprosy reactions (type 1, type 2 and/or acute neuritis) prior to US. The ulnar (at the cubital tunnel-Ut-and proximal to the tunnel-Upt), median (M) and common fibular (CF) nerves were scanned to measure cross-sectional areas (CSAs) in mm2 and to calculate the asymmetry indexes ΔCSA (absolute difference between right and left CSAs) and ΔUtpt (absolute difference between Upt and Ut CSAs). MB patients showed greater (p<0.05) CSAs than PB at Ut (13.88±11.4/9.53±6.14) and M (10.41±5.4/6.36±0.84). ΔCSAs and ΔUtpt were similar between PB and MB. The CSAs, ΔCSAs and ΔUtpt were similar between PB patients with reactions compared to PB patients without reactions. MB patients with reactions showed significantly greater CSAs (Upt, Ut and M), ΔCSAs (Upt and Ut) and ΔUtpt compared to MB patients without reactions. PB and MB showed similar frequencies of abnormal US measurements. Patients with reactions had higher frequency of nerve thickening and similar frequency of asymmetry to those without reactions. CONCLUSIONS/SIGNIFICANCE: This is the first study to investigate differences in nerve involvement among leprosy classification groups using US before treatment. The magnitude of thickening was greater in MB and in patients with reactions. Asymmetry indexes were greater in patients with reactions and did not significantly differ between PB and MB, demonstrating that asymmetry is a characteristic of leprosy neuropathy regardless of its classification.

Pure neuritic leprosy presenting as ulnar nerve neuropathy: a case report of electrodiagnostic, radiographic, and histopathological findings.

Hansen's disease, or leprosy, is a chronic infectious disease with many manifestations. Though still a major health concern and leading cause of peripheral neuropathy in the developing world, it is rare in the United States, with only about 150 cases reported each year. Nevertheless, it is imperative that neurosurgeons consider it in the differential diagnosis of neuropathy. The causative organism is Mycobacterium leprae, which infects and damages Schwann cells in the peripheral nervous system, leading first to sensory and then to motor deficits. A rare presentation of Hansen's disease is pure neuritic leprosy. It is characterized by nerve involvement without the characteristic cutaneous stigmata. The authors of this report describe a case of pure neuritic leprosy presenting as ulnar nerve neuropathy with corresponding radiographic, electrodiagnostic, and histopathological data. This 11-year-old, otherwise healthy male presented with progressive right-hand weakness and numbness with no cutaneous abnormalities. Physical examination and electrodiagnostic testing revealed findings consistent with a severe ulnar neuropathy at the elbow. Magnetic resonance imaging revealed diffuse thickening and enhancement of the ulnar nerve and narrowing at the cubital tunnel. The patient underwent ulnar nerve decompression with biopsy. Pathology revealed acid-fast organisms within the nerve, which was pathognomonic for Hansen's disease. He was started on antibiotic therapy, and on follow-up he had improved strength and sensation in the ulnar nerve distribution. Pure neuritic leprosy, though rare in the United States, should be considered in the differential diagnosis of those presenting with peripheral neuropathy and a history of travel to leprosy-endemic areas. The long incubation period of M. leprae, the ability of leprosy to mimic other conditions, and the low sensitivity of serological tests make clinical, electrodiagnostic, and radiographic evaluation necessary for diagnosis. Prompt diagnosis and treatment is imperative to prevent permanent neurological injury.

Leprosy: a 'common' and curable cause of peripheral neuropathy with skin lesions.

Leprosy (or Hansen's disease) is a curable chronic infectious disease caused by the acid-fast bacillus Mycobacterium leprae. While leprosy remains one of the most common causes of neuropathy worldwide, its rarity in the UK means that many doctors are unfamiliar with the typical clinical features. This is problematic because early recognition and treatment is vital in order to minimise disease-related complications such as nerve injury. We describe a 75-year-old man who presented with multiple mononeuropathy (mononeuritis multiplex, particularly affecting the ulnar nerves) and typical granulomatous skin lesions, in whom the diagnosis was made on the basis of skin biopsy. We highlight the clinical features, investigations and treatment of the patient, and provide information about the epidemiology and pathogenesis of leprosy.

[Linear tuberculoid leprosy along the lines of Blaschko: a very rare presentation]. / Lèpre tuberculoïde de disposition linéaire blaschkoïde: une présentation exceptionnelle.

BACKGROUND: Leprosy continues to be present in certain regions throughout the world, and the dermatologist plays a central role in its diagnosis. Herein we report a case of tuberculoid leprosy that is atypical in terms of its linear presentation which appears to follow the lines of Blaschko. PATIENTS AND METHODS: A patient from Mayotte was referred to the neurological department for suspected tuberculoid leprosy. He was presenting a deficiency of the ulnar nerve together with neuronal hypertrophy and cutaneous involvement. Dermatological examination revealed linear hypo-aesthetic hypopigmented lesions on the arm and forearm. The atypical clinical presentation also suggested to us pigmented mosaicism or post-inflammatory pigmentation. The biopsy showed granulomatous epithelial dermatitis with perinervous involvement. Imaging examinations confirmed the presence of neuronal hypertrophy. A diagnosis of linear tuberculoid leprosy was made. DISCUSSION: This case illustrates the need to bear in mind a diagnosis of leprosy in the event of hypopigmented lesions, even where they are linear and of Blaschkoid appearance. Several potential hypotheses may account for this particular topographical pattern.

Primary Neuritic Hansen's Disease presenting as Ulnar Nerve Abscess in a Human Immunodeficiency Virus Positive Patient.

Leprosy has been increasingly known to have an enigmatic relationship with human immunodeficiency virus infection. Co-infection may result in atypical manifestations of leprosy. A 45-year old human immunodeficiency virus-positive male; agricultural laborer presented with a swelling over right elbow, right hand deformity, generalized itching and recurrent vesicles overthe perinasal area. Clinical and investigational findings were consistent with mononeuritic type of Hansen's disease with right sided silent ulnar nerve abscess, partial claw hand. CD4+ count of the patientwas 430 cells/cmm. This patient also hadherpes simplex labialis, with HIV-associated pruritus. To the best of our knowledge such an atypical presentation has not been reported earlier.

Extensive ulnar nerve necrosis: a complication of tuberculoid leprosy.

Pure neural leprosy without cutaneous manifestations is a relatively rare manifestation of leprosy. It can present as a mono- or poly-neuritis with sensory and/or motor impairment. Neural leprosy may or may not be associated with thickening of the involved nerve. We report the case of a 14 year old boy with extensive ulnar nerve necrosis who was diagnosed to have tuberculoid leprosy. What makes this case unique is that we have here a case of pure neural leprosy with a single nerve turned 'necrotic'.

Clinically unsuspected neuritic leprosy with caseation necrosis.

Leprosy is a devastating disease caused by Mycobacterium leprae. It includes a spectrum of clinicopathological lesions. Neuritic leprosy with caseation necrosis (abscess) manifesting as a soft tissue mass is a relatively rare presentation of leprosy. Here, the authors report their experience with three patients with neuritic leprosy. The patients presented with swellings in the right ulnar nerve, the right great auricular nerve, and the temporal branch of the right sixth cranial nerve. The clinical impression was that of tumorous masses. Gross examination of the biopsy specimens revealed caseous necrotic materials. Further histological evaluation disclosed tuberculoid granulomas with extensive caseation necrosis. Stains for acid-fast bacilli were positive in the third case. A comparison between the caseation encountered in the tuberculoid neurotic leprosy and the neurolysis of lepromatous neurotic leprosy has been also discussed. The findings here emphasize "mass lesion with necrosis" as a possible clinical presentation of the neuritic leprosies. The clinicopathologic features were addressed and the relevant literature was reviewed.

Morphological changes of the epineurium in leprosy: a new finding detected by high-resolution sonography.

INTRODUCTION: From histopathological studies of peripheral nerves in leprosy, it is known that the epineurium can be thickened. We measured the epineurial thickness of the ulnar nerve by high resolution sonography (HRUS). METHODS: The epineurium of the ulnar nerve was measured above the elbow on transverse scan in 25 healthy controls and 26 leprosy patients. RESULTS: The mean epineurial thickness was 0.77 mm (95% confidence interval [CI] 0.66-0.88) in symptomatic ulnar nerves (n = 20), 0.58 mm (CI 0.51-0.65) in asymptomatic nerves (n = 30), and 0.49 mm (CI 0.44-0.54) in healthy controls (n = 25) (P = 0.0001). This thickening was related to the cross-sectional area of the ulnar nerve, but not with increased blood flow. CONCLUSIONS: The epineurium of the ulnar nerve can be measured with the use of HRUS, and it is often strikingly thickened in leprosy patients, especially in those with ulnar involvement.

Neural relapse in multibacillary leprosy 6 years after end of treatment.

This article presents a case of relapse, with isolated neural manifestation, in a multibacillary patient previously treated with multidrug therapy for multibacillary leprosy (24 doses). The patient returned to the service six years after the end of treatment, with pain in hands and legs. He was investigated, and the serological monitoring showed an important increase in anti-phenolic glycolipid serum levels. A neural recurrence was suspected, since the patient had no new skin lesions. A new biopsy in the right ulnar nerve showed a bacilloscopy of 2 +, compatible with relapse. This is a literature review of the etiological, clinical, propedeutical and diagnostic aspects of this situation so poorly understood.

Neural relapse in multibacillary leprosy 6 years after end of treatment / Recidiva neural em hanseníase multibacilar após 6 anos de tratamento

This article presents a case of relapse, with isolated neural manifestation, in a multibacillary patient previously treated with multidrug therapy for multibacillary leprosy (24 doses). The patient returned to the service six years after the end of treatment, with pain in hands and legs. He was investigated, and the serological monitoring showed an important increase in anti-phenolic glycolipid serum levels. A neural recurrence was suspected, since the patient had no new skin lesions. A new biopsy in the right ulnar nerve showed a bacilloscopy of 2 +, compatible with relapse. This is a literature review of the etiological, clinical, propedeutical and diagnostic aspects of this situation so poorly understood.

O presente artigo relata um caso de recidiva, com manifestação neural isolada, em paciente multibacilar previamente tratado com poliquimioterapia para multibacilar 24 doses. O paciente retorna ao serviço, seis anos depois do fim do tratamento, com dores em mãos e pernas. Na investigação, o acompanhamento da sorologia anti-glicolipídeo fenólico 1 demonstrou aumento importante dos níveis séricos, e foi aventada a hipótese de recidiva neural, já que o paciente não apresentava lesões cutâneas novas. Uma nova biópsia, em nervo ulnar direito, demonstrou baciloscopia de 2+, compatível com recidiva. Faz-se revisão da literatura sobre aspectos etiológicos, clínicos, propedêuticos e diagnósticos dessa situação tão pouco compreendida.