Sensory Ganglionopathy in Hansen’s Disease: Report of a Patient and Review of Literature

Hansen’s neuropathy usually affects cooler parts of the body, but presentation with poly-ganglionopathy is rare. We report a patient with ganglionopathy associated with pure neuritic Hansen’s disease and discuss it in the light of the reported literature. A 42-year-old male presented with painful distal paraesthesia and ataxia for three months. His sensory nerve conduction was unrecordable. Thyroid hormones, vasculitis profile, HIV serology and hepatitis workup were normal. MRI of nerves and whole-body positron emission tomography were also normal. Slit smear examination of the affected skin after appropriate staining and microscopic examination showed the presence of AFB (M. leprae). He was treated with rifampicin, clofazimine, dapsone and prednisolone, and at six-month follow-up, he was asymptomatic. We conclude that leprosy should be considered in the differential diagnosis of sensory poly-ganglionopathy, especially in tropical countries, as it is amenably treatable

Ganglionopatía sensitiva. Caso clínico y revisión de la bibliografía

Resumen: Las ganglionopatías sensitivas son un grupo raro de neuropatías sensitivas que pueden estar asociadas a neoplasias como el cáncer broncopulmonar. Su presentación clínica y resultado de estudio eléctrico son característicos. El tratamiento depende de la causa subyacente, siendo fundamental su hallazgo oportuno, ya que el inicio precoz impacta en el pronóstico del paciente. Reportamos el caso de una paciente que se presentó con parestesias de distribución atípica, dolor neuropático y ataxia. El estudio eléctrico evidenció una ganglionopatía sensitiva, de cuyo estudio etiológico surge el diagnóstico de carcinoma broncopulmonar.

Abstract: Sensory ganglionopathies are a rare group of sensory neuropathies that can be associated with neoplasms such as bronchopulmonary cancer. Its clinical presentation and electrical study result are characteristic. Treatment depends on the underlying cause, its timely finding being essential, since early onset impacts the patient's prognosis. We report the case of a patient who presented with atypical distribution paresthesias, neuropathic pain, and ataxia. The electrical study revealed a sensitive ganglionopathy, from whose etiological study arises the diagnosis of bronchopulmonary carcinoma.

Resumo: As ganglionopatias sensoriais são um grupo raro de neuropatias sensoriais que podem estar associadas a neoplasias como o câncer broncopulmonar. A apresentação clínica e o resultado do estudo elétrico são característicos. O tratamento depende da causa subjacente e seu achado oportuno é essencial, uma vez que o início precoce impacta o prognóstico do paciente. Relatamos o caso de um paciente que apresentou parestesias de distribuição atípica, dor neuropática e ataxia. O estudo elétrico revelou uma ganglionopatia sensível, de cujo estudo etiológico surge o diagnóstico de carcinoma broncopulmonar.

Neuronopatía sensitiva: Su reconocimiento y tratamiento temprano / Sensory neuronopathy. Its recognition and early treatment

Las neuronopatías o ganglionopatías sensitivas, o enfermedades del ganglio dorsal, representan un subgrupo de enfermedades del sistema nervioso periférico, frecuentemente asociadas a trastornos disinmunes o paraneoplásicos, y a agentes tóxicos. Los pacientes típicamente presentan ataxia temprana, pérdida de los reflejos osteotendinosos y síntomas sensitivos positivos, presentes tanto en partes proximales como distales del cuerpo. Estudiamos retrospectivamente 10 casos con un diagnóstico final de neuronopatía sensitiva. El síntoma de presentación fue el de una neuropatía sensitiva de curso subagudo en todos los casos, con parestesias en el 100% de los casos. Otras manifestaciones fueron: hipoestesia (10/10), ataxia de la marcha (8/10), síntomas autonómicos (3/10) y parestesias periorales (3/10). La electrofisiología mostró un patrón de compromiso sensitivo axonal, con respuestas motoras normales. El diagnóstico final fue neuronopatía sensitiva adquirida en todos, asociada a síndrome de Sjögren en dos, a lupus eritematoso en uno, a artritis reumatoidea en uno, a cáncer en dos (paraneoplásica) e idiopática en cuatro. En los casos paraneoplásicos, los tumores fueron un carcinoma de pulmón de células pequeñas (con anticuerpos anti-Hu positivos) y un carcinoma epidermoide de pulmón. Ocho pacientes fueron tratados con inmunoterapia, con altas dosis de metilprednisolona endovenosa y/o con inmunoglobulina endovenosa; con pobre respuesta en cuatro casos, mejoría neurológica en cinco, y sin cambios en uno. El presente trabajo muestra el patrón clinico y electrofisiológico de las neuronopatías sensitivas subagudas, y la relevancia de un tratamiento temprano.

Sensory neuronopathies or ganglionopathies, or dorsal root ganglion disorders, represent a subgroup of peripheral nervous system diseases, frequently associated with dysinmune or neoplastic disorders and with toxic agents. A degeneration of both central and peripheral sensory proyections is present. Patients typically show early ataxia, loss of deep tendon reflexes and positive sensory symptoms present both in proximal and distal sites of the body. We retrospectively studied 10 cases with a final diagnosis of sensory neuronopathy. Sensory neuropathy was the presenting symptom and the course was subacute in all cases. Paresthesias in upper limbs were a predominant manifestation (100%). Other manifestations included: hypoesthesia (10/10), gait ataxia (8/10), autonomic symptoms (3/10) and perioral paresthesias (3/10). Electrophysiology showed sensory axonal neuronal pattern, with normal motor responses. Final diagnosis was acquired sensory neuronopathy in all patients, associated with Sjögren’s syndrome in 2, with lupus erythematosus in 1, with rheumatoid arthritis in 1, with a cancer in 2 (paraneoplastic) and idiopathic in 4. In paraneoplastic cases, the tumor was small cell lung cancer in 1 (with positive anti-Hu antibodies), and epidermoid lung cancer in the other. Eight patients were treated with immunotherapy, high dose intravenous methylprednisolone and/or intravenous immunoglobulin; with poor response in 4 cases, neurologic improvement in 5, and without any change in 1 patient. The present work shows the typical clinical and electrophysiological pattern of subacute sensory neuronopathy, and the relevance of early treatment.

Neurogenic Bladder Dysfunction Caused by Seronegative Autoimmune Autonomic Ganglionopathy

Autoimmune autonomic ganglionopathy is a form of acquired autonomic failure affecting parasympathetic, sympathetic functions, usually affecting healthy young people. The disorder affects both sympathetic and parasympathetic nervous systems, with acute onset, monophasic course, and partial recovery with relative preservation of motor and sensory function. We experienced a case of young man with acute autoimmune autonomic ganglionopathy who developed voiding difficulty, sudden blurred vision and gastrointestinal discomfort without motor or sensory dysfunction. Fever developed 5 days earlier and persisted until onset of autonomic failure. Patient complained voiding difficulty and urodynamic study revealed detrusor are flexia with failure to initiate and sustain adequate detrusor contraction. Sympathetic skin response and several autonomic function tests showed abnormalities. Intravenous immunoglobulin was applied for 5 days but symptoms persisted. Thus, 5 days of plasmapheresis treatment was followed showing improvements in most of the symptoms. However bladder dysfunction persisted at 6 months follow-up, showing partial recovery at bethanechol administration.

Ulnar sensory-motor amplitude ratio: a new tool to differentiate ganglionopathy from polyneuropathy / Razao de amplitude sensitivo-motora ulnar: novo parametro para diferenciar ganglionopatia de polineuropatia

The objective of this study was to evaluate if the ratio of ulnar sensory nerve action potential (SNAP) over compound muscle action potential (CMAP) amplitudes (USMAR) would help in the distinction between ganglionopathy (GNP) and polyneuropathy (PNP). Methods We reviewed the nerve conductions studies and electromyography (EMG) of 18 GNP patients, 33 diabetic PNP patients and 56 controls. GNP was defined by simultaneous nerve conduction studies (NCS) and magnetic resonance imaging (MRI) abnormalities. PNP was defined by usual clinical and NCS criteria. We used ANOVA with post-hoc Tukey test and ROC curve analysis to compare ulnar SNAP and CMAP, as well as USMAR in the groups. Results Ulnar CMAP amplitudes were similar between GNP x PNP x Controls (p=0.253), but ulnar SNAP amplitudes (1.6±3.2 x 11.9±9.1 × 45.7±24.7) and USMAR values (0.3±0.3 × 1.5±0.9 × 4.6±2.2) were significantly different. A USMAR threshold of 0.71 was able to differentiate GNP and PNP (94.4% sensitivity and 90.9% specificity). Conclusions USMAR is a practical and reliable tool for the differentiation between GNP and PNP. .

O objetivo deste estudo foi avaliar se a razão entre as amplitudes dos potenciais de ação sensitivo (SNAP) e motor (CMAP) do nervo ulnar (USMAR) auxiliaria na distinção entre ganglionopatia (GNP) e polineuropatia (PNP). Métodos Revisamos os estudos de neurocondução e eletromiografia de 18 pacientes com GNP, 33 com PNP diabética e 56 controles. GNP foi definida pela presença simultânea de anormalidades na neurocondução e na ressonância magnética cervical. PNP foi definida por critérios clínicos e neurofisiológicos usuais. Usamos o teste ANOVA com Tukey post-hoc e análise da curva ROC para comparar o SNAP e CMAP ulnares, assim como o USMAR entre os grupos. Resultados As amplitudes dos CMAPs ulnares foram similares entre GNP × PNP × Controles (p=0,253), mas as amplitudes dos SNAPs ulnares (1,6±3,2 × 11,9±9,1 × 45,7±24,7) e os valores de USMAR (0,3±0,3 × 1,5±0,9 × 4,6±2,2) foram significativamente diferentes. Um corte de 0,71 para a USMAR foi capaz de diferenciar GNP de PNP (sensibilidade de 94,4% e especificidade de 90,9%). Conclusões A USMAR é um parâmetro útil e confiável para o diagnóstico diferencial entre GNP e PNP. .