Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report

The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an unusual but important complication of hematopoietic stem cell transplantation (HSCT) rarely reported to date. We describe a 17-year-old woman with a diagnosis of acute myeloid leukemia due to Fanconi's anemia who was submitted to allogeneic HSCT and developed CIDP as part of graft-versus-host disease. Investigation showed high cerebrospinal fluid protein; electrophysiological studies revealed sensory-motor demyelinating polyradiculoneuropathy; muscle and nerve biopsy were compatible with CIDP.

A polirradiculoneuropatia desmielinizante inflamatória crônica (CIDP) é uma incomum, porém, importante complicação do transplante de células hematopoiéticas (HSCT) raramente relatada até a data. Nós descrevemos uma mulher de 17 anos com diagnóstico de leucemia mielóide aguda por anemia de Fanconi que foi submetida à HSCT e desenvolveu CIDP como parte da doença do enxerto contra o hospedeiro. A investigação mostrou elevação na proteína no líquor; estudo eletrofisiológico revelando polirradiculoneuropatia desmielinizante sensitivo-motora; e biópsia de músculo e nervo compatível com CIDP.

Neural toxicity induced by accidental intrathecal vincristine administration

Described here is a case of accidental intrathecal administration of vincristine with pathologic findings in the central nervous system. A 3-year-old boy with acute lymphoblastic leukemia, was given his ninth course chemotherapy. Vincristine was accidentally injected intrathecally. The clinical course was rapidly progressive (6-day course) and resulted in death. An autopsy was done. The brain and spinal cord was grossly edematous and congested without any specific feature. Histologically, profound loss of neuron was noted in the spinal cord. Remaining neurons in the spinal cord, particularly anterior horn cells were markedly swollen. The spinal nerves show diffuse axonal degeneration and myelin loss. The upstream portion of the spinal cord (brain stem, cerebellum, cerebrum) showed patchy loss of neurons, especially Purkinje cells and granular cells of the cerebellar cortex. Many neurons showed axonal reaction (chromatolysis) with swelling. Several neurons show intracytoplasmic eosinophilic inclusion body. Myelin loss, axonal swelling and enlargement of perivascular spaces were seen throughout the white matter of central nervous system.

Enfermedad de Landry-Guillain-Barre-Strohl: estudio clínicopatológico de 35 casos de autopsia / Landry-Guillain-Barre-Strohl disease: a clinical and pathological study of 35 autopsy cases

Se informa una serie de 35 casos autopsiados de polineuropatía inflamatoria idiopática aguda (Enfermedad de Guillain-Barré). La enfermedad fué más frecuente en hombres, principalmente en los decenios tercero y quinto de la vida. En lamayoría de los enfermos no xistió una infección viral previa y la elevación de proteínas en el líquido cerebro espinal fué sólo un hallazgo ocasional. El tiempo de evolución fué muy variable y el síntoma predominante fué déficit motor. Los hallazgos neuropatológicos fueron microscópicos, desde muy sutiles como edema, desmielinización de raíces motoras y activación de células de Schwann, hasta cromatólisis central en las neuronas de las astas anteriores de la médula espinal, indicaativa de daño axonal en casos de evolución de más de 10 días. Se comparan los datos obtenidos en nuestro estudio con los de otras series y se discute la probable patogenia de la entidad

Diagnostic usefulness and limitations of the sural nerve biopsy

In recent years, the sural nerve biopsy has become a commonly performed procedure in the diagnostic work-up of patients with peripheral neuropathy. This paper reviews the diagnostic usefulness and limitations of this procedure. Based on 385 sural nerve biopsies, we found clinically helpful or relevant information in 45% of cases. In 24% of cases, specific diagnoses were obtained, among which vasculitic neuropathy was most common.