1/1046. Chronic motor axonal neuropathy: pathological evidence of inflammatory polyradiculoneuropathy. Chronic immune and inflammatory motor neuropathies may resemble motor neuron disease, and the distinction may be particularly difficult if conduction block or GM1 antibodies are absent. The pathology of this axonal type of chronic motor neuropathy has not been characterized except in a few cases associated with paraproteinemia. We describe the clinical, electrophysiological, and pathological findings in a patient with a chronic motor axonal neuropathy, normal immunoelectrophoresis, and no GM1 antibodies. At autopsy the spinal cord was normal with the exception of chromatolytic motor neurons. All the ventral roots were greatly thinned. Of 10 mixed nerves and numerous spinal roots sampled, five showed areas of perineurial, perivascular lymphocytic infiltration. There was severe axonal loss in the motor roots that was not as evident in mixed nerves, and the sensory nerves and roots were virtually unaffected. Our findings suggest that a chronic motor axonal neuropathy without paraproteinemia or GM1 antibodies may, in some cases, result from an inflammatory process. ( info) |
2/1046. Cortical dysplasia with angiodysgenesis and chronic inflammation in multifocal partial epilepsy. A 25-year-old man with a long history of temporal lobe epilepsy developed right occipital lobe seizures and a progressive right homonymous hemianopia. MRI showed diffuse enhancement of the left temporoparieto-occipital white matter and cortical thickening of the left medial temporal lobe. The resected temporal lobe revealed cortical dysplasia and angiodysplasia with foci of more recent ischemic necrosis and chronic inflammation as an explanation for the clinical deterioration. ( info) |
3/1046. Chronic axonal sensory and autonomic polyneuropathy without motor involvement: a new 'chronic inflammatory neuropathy?'. We report the case of a woman with axonal sensory and autonomic neuropathy lasting several months who improved in association with steroid administration. During the course of her disease and in the follow-up, the patient underwent repeated cerebrospinal fluid (CSF) examinations, neurophysiological somatic, autonomic nervous system studies and sural nerve biopsy. Clinical and laboratory assessments demonstrated the occurrence of a monophasic, chronic sensory and autonomic neuropathy. A sural nerve biopsy suggested an axonopathy. After a progressive worsening of symptoms lasting about 6 months, steroid treatment was started and within 6 months a complete recovery, with normalization of the CSF findings, was observed. Although the 'chronic inflammatory neuropathies' are still debated entities, the features of this chronic, exclusively sensory and autonomic neuropathy are new, and the occurrence of a high protein level in the CSF, together with the favorable outcome associated with steroid treatment, suggests that our case might be another variant in this debated area. ( info) |
4/1046. Histologically proven lymphocytic hypophysitis: spontaneous resolution and subsequent pregnancy. Of the 128 previously reported cases of lymphocytic hypophysitis, the diagnosis was histologically proven in 6 patients in whom the pituitary mass regressed spontaneously; only 1 subsequently became pregnant. Among six additional patients who became pregnant after a diagnosis of presumed lymphocytic hypophysitis, the disease was confirmed histologically in only three, two of whom underwent surgical debulking and one who had no follow-up imaging. To our knowledge, we describe the second patient with histologically proven lymphocytic hypophysitis, associated with adrenocorticotropic hormone (corticotropin) and prolactin deficiencies, in whom the pituitary mass regressed completely with physiologic hydrocortisone therapy only and in whom a spontaneous pregnancy occurred subsequently with no postpartum recurrence of the pituitary mass. This information lends credence to previous recommendations that, in the absence of visual field defects, surgical and corticosteroid therapy may be safely withheld, with periodic reassessment. ( info) |
| The authors report an association between acute inflammatory neuropathy and previously undiagnosed Charcot-Marie-Tooth 1A disease in a 15-year-old girl. sural nerve biopsy study showed hypertrophic neuropathy with endoneurial infiltrates of macrophages and lymphocytes. This association may be coincidental, but a particular susceptibility to damage of these peripheral nerves cannot be excluded. This report confirms the importance of pes cavus as a sign of long-standing sensorimotor neuropathy. ( info) |
6/1046. Inflammatory metastatic melanoma. An 87-year-old woman developed erythema, induration and tenderness of the skin overlying each breast. One year before, she had undergone an axillary lymph node dissection because of metastases from melanoma. The primary site was unknown. A skin biopsy showed pigmented tumor nests within the dermal lymphatic vessels, and immunohistochemistry confirmed the melanocytic origin. The diagnosis of inflammatory metastatic melanoma was made. ( info) |
7/1046. Probable lymphocytic hypophysitis diagnosed by short-term serial computed tomography and gallium-67 scintigraphy--case report. A 61-year-old female presented with headache, malaise, and left oculomotor nerve paralysis. Computed tomography (CT) demonstrated a diffuse pituitary mass and enlarged pituitary stalk with homogeneous contrast enhancement. Her symptoms gradually resolved without treatment. gallium-67 scintigraphy showed abnormal uptake in the pituitary lesion. Serial CT every 2 weeks after admission showed homogeneous contrast enhancement and shrinking of the pituitary mass to a normal size 12 weeks after the onset. The final diagnosis was lymphocytic adenohypophysitis without biopsy. recurrence has not been observed for 8 years after discharge. The patient did not need hormone replacement therapy. Histological examination is not always necessary to diagnose probable lymphocytic adenohypophysitis with the characteristic feature of rapid onset, abnormal gallium-67 uptake in the lesion, and resolution of symptoms in the acute stage with shrinking of the lesion on neuroimaging. ( info) |
8/1046. Fine-needle aspiration cytology of well-differentiated inflammatory liposarcoma: a case report with histologic follow-up. Inflammatory liposarcoma is an unusual variant of well-differentiated liposarcoma. We report on the fine-needle aspiration (FNA) cytology findings of a retroperitoneal well-differentiated inflammatory liposarcoma from a 63-yr-old white female. The smears showed numerous dispersed inflammatory cells, with the majority being reactive lymphoid cells and plasma cells. There were scattered, large, atypical cells containing multiple or hyperlobated nuclei with coarse chromatin and abundant ill-defined cytoplasm. The large atypical cells, as well as the inflammatory cells, were also found within the fibrous tissue fragments. The follow-up surgical resection of the tumor demonstrated a well-differentiated inflammatory liposarcoma with coexistent dedifferentiated areas and lipoma-like, well-differentiated liposarcoma. With the appropriate anatomic and radiographic settings, the FNA cytology findings of abundant, reactive inflammatory cells and scattered, large, atypical tumor cells that are CD15-, CD30-, and cytokeratin-negative are highly suggestive of a well-differentiated inflammatory liposarcoma. ( info) |
9/1046. Autoaggressive inflammation of the myenteric plexus resulting in intestinal pseudoobstruction. After a 3-year history of severe constipation, a 16-year-old girl required surgery to be relieved of impacted stools. Histologic examination showed ganglionitis in the myenteric plexus of the large bowel and ileum, whereas the submucosal plexus was spared. At this time, antineuronal nuclear antibodies (ANNA-1, anti-Hu) were found at high titer in the serum of the patient. One and a half years earlier, a paravertebral ganglioneuroblastoma had been removed. Histologic examination had shown undifferentiated neuroblasts and morphologically mature ganglion cells with both cell types embedded in an inflammatory infiltrate morphologically similar to the lymphoplasmocytic infiltration seen in the myenteric plexus. The patient's serum was found to bind to nuclei of mouse intestinal tract neurons, thus fulfilling defining criteria for ANNA-1. The serum also reacted with antigens of defined molecular weight in a Western blot, thus fulfilling defining criteria for anti-Hu. Expression of the Huantigen could be visualized in the nuclei of the patient's tumor cells by immunohistochemistry. These tests showed that an antitumor inflammatory response was the cause of the bowel disease. This is the first report of a tumor from the neuroblastoma group that caused paraneoplastic intestinal pseudoobstruction. Ganglionitis and subsequent aganglionosis are the hallmark of the morphologic diagnosis which cannot be obtained by suction biopsy in patients with intact submucosal plexus. Instead, serum testing for autoantibodies can reveal the etiology. ( info) |
10/1046. Conjunctival melanocytic nevi of childhood. Two young patients with conjunctival compound nevi are presented to illustrate two types of abnormalities that lead to difficulty in distinction of these nevi from invasive melanomas. In Case 1, inflammation is associated with disruption of the nevus cell architecture and cytologic atypia. In Case 2, the occurrence of a combined nevus (compound and blue nevus types) in the conjunctiva leads to diagnostic problems. Circumscription of the lesions, lack of mitoses in the substantia propria, and lack of pagetoid spread of atypical cells in the adjacent conjunctival epithelium support benign diagnoses in both cases. ( info) |