Long-term effects of pancreatic islet transplantation on polyneuropathy in patients with brittle diabetes: A single-center experience.

INTRODUCTION/AIMS: Pancreatic islet transplantation (ITx) is increasingly used in patients with brittle type 1 diabetes (T1D). If successful, ITx results in insulin-free euglycemia, but its application is limited by a need for lifelong immunosuppression. The aim of this study was to assess the long-term effects of ITx on the occurrence and course of polyneuropathy in a cohort of patients with brittle T1D. METHODS: In this prospective, single-center study, 13 patients (4 males and 9 females) with brittle T1D had a baseline neurological exam with the calculation of Utah Neuropathy Scale (UNS) and a limited nerve conduction study before ITx, and about yearly after in the patients who achieved insulin independence. RESULTS: Patients were followed for a period of 17 to 133 months. There was no significant difference between UNS and nerve conduction study parameters at baseline and at the end of follow-up, except for significant decreases in peroneal (50.34 ± 6.12 vs. 52.42 ± 6.47 ms, P = 0.005) and ulnar (27.5 ± 2.15 vs. 29.45 ± 2.10 ms, P = 0.009) F-wave latencies and an increase in ulnar sensory nerve conduction velocity (49.98 ± 6.27 vs. 47.19 ± 5.36 m/s, P = 0.04). DISCUSSION: If successful, ITx has a good long-term safety profile for peripheral nerve toxicity, and a favorable effect on diabetic neuropathy.

Acute encephalopathy as the initial manifestation of CADASIL.

A 29-year-old right-handed G1P1 Caucasian woman presented with acute bifrontal headache (which resolved within 1 day), confusion, and difficulty using her right hand on postpartum day 10. She did not report nausea, vomiting, or visual complaints. The patient was previously healthy except for her recent preeclampsia, which required emergent cesarean section. On examination, the patient was afebrile, awake, alert, and apathetic. She was able to follow few one-step midline commands (e.g., eye opening and closing) inconsistently but not appendicular commands. Her neurologic deficits were remarkable for expressive aphasia, intermittent receptive aphasia, and hyperreflexia with bilateral extensor plantar responses. No meningismus or other focal neurologic deficits were present. Routine laboratory testing including urine toxicology screen was normal. C-reactive protein was 23 mg/L (reference range: <5 mg/L), and erythrocyte sedimentation rate (ESR) was 38 mm/h (reference range: 0-20 mm/h). Rheumatologic panel was negative. Brain MRI showed extensive non-contrast-enhancing T2/fluid-attenuated inversion recovery hyperintensities involving periventricular and deep white matter, especially the centrum semiovale, corpus callosum, bilateral anterior temporal lobes, bilateral caudate nucleus, and globus pallidus (figure, A-C). No evidence of acute or previous stroke was found. CT angiogram and venogram revealed no cerebral sinus thrombosis or large vessel vasculitis. Lumbar puncture opening pressure was 18.5 cm H2O. CSF showed normal cell counts, protein, and glucose levels without oligoclonal bands. EEG recorded in awake, drowsy, and sleep state was normal. Dilated ophthalmic examination showed no microangiopathy or retinal branch arterial occlusion. Audiologic examination was normal.

Polytetrafluoroethylene-induced granuloma and brainstem cyst after microvascular decompression for trigeminal neuralgia: case report.

OBJECTIVE: Microvascular decompression is commonly performed for medically refractory trigeminal neuralgia. A piece of polytetrafluoroethylene (PTFE) is usually placed between the trigeminal nerve and the blood vessel causing the compression. The procedure is effective and relatively safe, and PTFE is presumed to be inert. Reactions to PTFE are rare. CLINICAL PRESENTATION: We report a patient who developed progressive neurological symptoms 5 years after microvascular decompression surgery. Imaging showed an enhancing cerebellopontine mass resembling a posterior fossa tumor with a large cyst compressing the brainstem. INTERVENTION: Craniotomy was performed to decompress the cyst. Biopsy of the enhancing mass showed granulomatous inflammation. The patient underwent a second brainstem decompression surgery with placement of a catheter in the cyst connected to an Ommaya reservoir; she has moderate to severe residual neurological deficits. CONCLUSION: This may be the first case of a severely disabling, space-occupying cyst resulting from a reaction to intracranial PTFE. Should this exceptionally rare complication be disclosed to patients or is it an idiosyncratic reaction unlikely to occur again?

Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor.

Malignant peripheral nerve sheath tumors are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest. They arise from the spinal roots, peripheral nerves, brachial and lumbosacral plexi, cranial nerves and terminal nerve fibers within soft tissue, intestine, lung and bone. These tumors recur either locally, or metastasize distally. Most of these tumors occur in association with neurofibromatosis type 1. Spinal cord metastasis from malignant nerve sheath tumors associated with neurofibromatosis type 1 is very rare. We describe a rare case of near-total spinal cord metastasis in a patient with malignant nerve sheath tumor in the absence of neurofibromatosis, and highlight the microscopic findings and natural history of this disease process.

Acute postoperative shingles after thoracic sympathectomy for hyperhidrosis.

Shingles secondary to reactivation of a previous varicella-zoster virus infection has been reported to develop within surgical wounds and after trauma. We report the case of a 17-year-old girl with history of chicken pox in childhood who had acute postoperative shingles develop along the T3-T4 dermatomes after thoracic sympathectomy for hyperhidrosis. The possible causes and precipitating factors are discussed.