Chronic inflammatory demyelinating polyneuropathy and ventilatory failure: report of seven new cases and review of the literature.

BACKGROUND: Ventilatory involvement is rarely reported in chronic inflammatory demyelinating polyneuropathy (CIDP), but small prospective studies showed frequent involvement of phrenic nerves, which is usually overshadowed by severe limb weakness. OBJECTIVES: To report the clinical features of CIDP associated with ventilatory failure. RESULTS: There were seven patients (43% women), with a mean age of 58.6 (range 38-82). The clinical courses were relapsing in five and progressive in two. Four patients had an initial event simulating Guillain-Barre syndrome (GBS). Ventilatory failure was recurrent in three patients. Five patients had full or nearly complete recoveries; one still requires nocturnal ventilation; and one died (14%) of myocardial infarction while still requiring mechanical ventilation. CONCLUSIONS: Clinical ventilatory dysfunction in CIDP is usually not an indicator of poor prognosis, and many patients recover without significant permanent disability. The mortality rate is similar to intubated patients with GBS. Patients with cardiopulmonary comorbidities and acute GBS-like onset of CIDP may be at higher risk of ventilatory failure which typically responds to 'standard' treatments of CIDP. Larger prospective studies are needed to define the prevalence, clinical spectrum and significance of ventilatory involvement in CIDP and to establish guidelines for evaluation and treatment.

Treatment of tardive dyskinesia with levetiracetam in a transplant patient.

OBJECTIVE: To describe successful treatment of tardive dyskinesia with levetiracetam. BACKGROUND: Tardive dyskinesia is a late-onset movement disorder caused by exposure to dopamine receptor blocking agents, most commonly neuroleptics. Metoclopramide is frequently used to treat gastrointestinal dysmotility. It has antidopaminergic properties, and is estimated to be responsible for two-thirds of drug-related movement disorders. DESIGN/METHODS: Case report. RESULTS: A 68-year-old woman presented with a history of intestinal transplantation (12 years ago; short gut syndrome related to bowel resection for rectal carcinoma) and renal transplantation (1 year ago; diabetes). She developed involuntary movements with stereotypic oro-buccal-lingual dyskinesias and right-sided choreiform movements. Her Abnormal Involuntary Movement Scale score (AIMS) score was 27. She has been treated with metoclopramide for gastrointestinal dysmotility for more than 10 years and was diagnosed with tardive dyskinesia. Treatment with levetiracetam 250 mg orally b.i.d. led to a significant improvement of abnormal movements within a week. Her AIMS score decreased to 8. DISCUSSION: Tardive dyskinesia may be quite disabling and options include withdrawal of offending medication, or use of tetrabenazine or reserpine. Several reports also suggested improvement of tardive movement disorders with levetiracetam. In our patient, levetiracetam relieved symptoms of tardive dyskinesia and allowed continuous use of metoclopramide. Larger studies are needed to confirm its efficacy.

Vasculitic neuropathy--electrodiagnostic findings and association with malignancies.

BACKGROUND: Vasculitic neuropathies occur in the context of systemic disorders or in isolation. Histopathologic evaluation remains the gold standard for diagnosis, but certain electrodiagnostic findings may heighten suspicion of vasculitic neuropathy and improve the yield of nerve and muscle biopsy. AIM OF THE STUDY: Description of electrodiagnostic patterns associated with vasculitic neuropathies, and a report of a possible association with malignancies. METHODS: Retrospective review of medical records of patients with histopathologically proven vasculitic and non-vasculitic axonal neuropathies evaluated at the University of Pittsburgh Medical Center from November 1995 to November 2003. RESULTS: The most distinctive electrodiagnostic patterns associated with vasculitic neuropathy were mononeuritis multiplex (27.5% vs 4% in controls; P = 0.003) and axonal sensorimotor polyneuropathy with side-to-side amplitude asymmetry (50% vs 32%, P > 0.05). Additionally, six patients (15% vs 2%; P = 0.034) developed various malignancies within 2 years of onset of vasculitic neuropathy. CONCLUSIONS: While generalized polyneuropathy was the most common presentation of nerve vasculitis, our study affirms side-to-side amplitude asymmetry and mononeuritis multiplex as the most distinctive electrodiagnostic features. The frequent occurrence of malignancies suggests a possible association with the vasculitic neuropathy and warrants additional investigation.

Granulomatous amebic encephalitis in a multivisceral transplant recipient.

A 40-year-old man with multivisceral allograft developed acutely right-sided numbness 9 months after transplantation. Cranial magnetic resonance imaging (MRI) showed a small left parietal lesion, and cerebrospinal fluid analysis was unremarkable. Stereotactic brain biopsy was non-diagnostic. The patient continued to deteriorate, developed cerebral edema and died at 13 days after the onset of symptoms. Unexpectedly, autopsy demonstrated acanthamebic encephalitis. This case highlights diagnostic difficulties encountered with amebic encephalitis and expands the spectrum of opportunistic central nervous system (CNS) infections in solid and visceral organ transplant recipients.

High titers of CA-125 may be associated with recurrent ischemic strokes in patients with cancer.

In addition to etiologies common in the general population, strokes in cancer patients may be caused by hypercoagulable states, hyperviscosity, cardiogenic embolism, and neoplastic vessel infiltration. Intravascular mucins were reported in patients with recurrent thromboembolism. The authors report four patients with metastatic cancer, brain infarcts, and other thromboembolic disease with markedly elevated levels of the tumor marker CA-125 and explore possible associations between this mucinous protein and strokes.

Subacute rhombencephalitis optica responsive to intravenous immunoglobulins.

A 53-year-old man presented with optic neuritis, followed within 10 weeks by a subacute progression of weakness, ataxia, and multiple cranial nerve palsies. Cranial magnetic resonance imaging demonstrated multiple T2-hyperintense lesions extending from the ponto-medullary junction to the thalamus and internal capsule. Cerebrospinal fluid analysis showed lymphocytic pleocytosis with elevated protein content. A brain biopsy revealed inflammatory changes. After a month long period of inexorable worsening, the patient was treated with intravenous immunoglobulins (2 g/kg). He responded to treatment, and recovered over the subsequent 3 months. The patient's response to treatment suggests that intravenous immunoglobulins should be considered in patients with inflammatory brain disorders.

Sensory neuropathy associated with metronidazole: report of four cases and review of the literature.

OBJECTIVE: To better characterize sensory neuropathy associated with metronidazole. METHODS: We report four patients who developed dysesthesias after metronidazole treatment. One received topical metronidazole only. All four underwent electrodiagnostic studies, including nerve conduction studies (NCS), quantitative sensory testing (QST), and quantitative sudomotor axon reflex testing (QSART). One underwent nerve biopsy. RESULTS: NCS were normal in all patients. QST showed impaired vibration thresholds in three patients. Three of four patients had abnormal tests of small fiber function. Cooling thresholds were abnormal in one; QSART was abnormal in one and borderline in another. The nerve biopsy specimen showed mild loss of small myelinated axons. CONCLUSIONS: This study shows that paresthesias associated with metronidazole exposure may be the result of a relatively mild sensory neuropathy with predominant involvement of small fibers and milder, mostly subclinical involvement of large fibers.

Rapidly progressive stroke in a young adult with very low high-density lipoprotein cholesterol.

Ischemic strokes can affect young adults (15-45 years old). Most such strokes are caused by cardioembolic events, small vessel disease, or illicit drug use, and less frequently by large vessel atherosclerosis. Large vessel cerebral atherosclerosis is usually associated with high levels of low-density lipoprotein (LDL) cholesterol, but a low level of high-density lipoprotein (HDL) is also a risk factor for ischemic strokes. The magnitude of increased risk is unclear, particularly with extremely low HDL levels found only in various genetic and inherited disorders. Advanced atherosclerosis developed in the patient in this study, with HDL of 3 mg/dL, leading to rapidly progressive stroke with a fatal outcome. The disease primarily affected the posterior circulation. The course of this case illustrates that very low HDL may be associated with advanced cerebrovascular atherosclerosis and fatal stroke, and as such should be considered in young individuals with stroke.