Substantia nigra hyperechogenicity in essential tremor and Parkinson's disease: a longitudinal study.

BACKGROUND AND PURPOSE: Essential tremor (ET) and Parkinson's disease (PD) sometimes overlap in their clinical expression with ET preceding PD onset, often leading to misdiagnosis. Transcranial sonography (TCS) has been shown to be a valid and non-invasive diagnostic tool to identify early idiopathic PD and to differentiate it from ET. The purpose of this study was to investigate the relevance of substantia nigra hyperechogenicity in patients with ET. METHODS: A total of 138 patients (79 with PD, 59 with ET) and 50 matched controls underwent TCS examination at baseline. All patients were followed in a 3-year longitudinal assessment. RESULTS: A total of 10 subjects were excluded from the analysis due to the bilateral absence of a temporal acoustic window. During the follow-up period, 11 of the patients with ET developed new-onset parkinsonian features, without fulfilling criteria for PD diagnosis (ET+). Nine patients developed clinical features meeting diagnostic criteria for probable PD (ET-PD). Patients with ET- did not develop parkinsonian features. For each group, the maximum size of the substantia nigra hyperechogenicity was as follows: 5.62 ± 5.40 mm2 in the control group, 19.02 ± 14.27 mm2 in patients with PD, 9.15 ± 11.26 mm2 in patients with ET-, 20.05 ± 13.78 mm2 in patients with ET+ and 20.13 ± 13.51 mm2 in patients with ET-PD. ET-PD maximum values were significantly different from controls. Maximum values in patients with ET+ were different from both controls and patients with ET-. CONCLUSION: Substantia nigra hyperechogenicity in ET seems to represent a risk marker for developing early parkinsonian symptoms or signs in the 3 years following TCS assessment.

[Amyotrophic lateral sclerosis in a patient with Waldenstrom's macroglobulinemia]. / Sclerosi laterale amiotrofica in un paziente con macroglobulinemia di Waldenstrom.

Although a peripheral neuropathy is the best known neurological complication of Waldenstrom's Macroglobulinemia (WM), the association of Amyotrophic Lateral Sclerosis (ALS), or other Motor Neuron Diseases (MND) with monoclonal gammopathies is described. We report the case of a male patient (41 years old) with WM and ALS. Whether monoclonal gammopathies play a role in the pathogenesis of MND is unclear but is la possible that patients might have antibodies against motor neurons. In our reported case neurologic symptoms were the first and the most important manifestations of the underlying hemopathy and despite plasmapheresis and immunosuppressive treatment ALS syndrome progressed. The neurologic disease worsened despite the improvement of WM.