Lymphangioleiomyomatosis (LAM) is a rare disease characterized by pulmonary cysts at computed tomography (CT) and proliferation of abnormal smooth muscle cells at lung biopsy. Almost all patients are female, and all have pulmonary cysts at high-resolution CT. Although the presence of cysts may be suggested at conventional CT or chest radiography, high-resolution CT is superior for cyst detection and is essential for diagnosis. The cysts are typically round; in most cases, the cyst wall is barely seen at thin-section CT. They are typically diffusely distributed throughout the central and peripheral lung parenchyma. The lung bases are affected in all patients. Some patients also have increased lung attenuation or a reticular pattern. Expiratory CT shows no air trapping between the cysts, and most of the cysts decrease in size. Pneumothorax, pleural effusion, and chylothorax are complications of LAM. Certain abdominal findings may provide additional corroborative evidence of the diagnosis. Renal angiomyolipomas, the most frequent abdominal lesions, usually manifest as asymptomatic, small, bilateral tumors of fat attenuation in the renal cortex. Lymphangiomas are cystic retroperitoneal masses that occur in up to 20% of patients. Other CT findings are hypo- or hyperattenuating lymph nodes, a dilated thoracic duct, and ascites.

We studied 30 patients whose primary complaint was head tremor in an attempt to characterize neurophysiological aspects of their abnormal movement. Based on family medical history and physical examination, 23 patients had definite or probable essential tremor (essential head tremor, EHT). The remaining seven had mild dystonic signs accompanying their head tremor (head tremor plus dystonic signs, HT + DS). We recorded head movement and the electromyographic (EMG) activity of the sternomastoid and splenius capitis muscles, determined the spontaneous blinking rate, and measured the excitability recovery curve of the blink reflex and of the masseteric inhibitory reflex. All patients had tremor bursts at a frequency ranging between 3 and 9 Hz in at least one of the muscles examined. The predominant pattern seen when patients were sitting relaxed and facing forward was that of synchronized EMG bursts in both splenius capitis muscles. Maintenance of extreme head postures demonstrated two types of additional abnormalities: type 1 (enhancement of tremor), which was observed in 11 patients (47.8%) with EHT and in two (28.5%) with HT + DS; and type 2 (activation of neck muscles not required for maintenance of the posture), which was observed in two patients (8.7%) with EHT and in five (71.5%) with HT + DS (chi 2 = 26.4; p < 0.001). Mean blinking rate per minute was 24.9 +/- 14.6 in patients with EHT and 42.3 +/- 10.5 in patients with HT + DS (paired t test, p = 0.001). The blink reflex and masseteric inhibitory reflex excitability recovery curves showed an abnormal interneuronal excitability enhancement in seven (30.4%) of the 23 patients with EHT and in two (28.5%) of the seven with HT + DS (chi 2 = 3.1; p > 0.05). Abnormal patterns of EMG activity of the neck muscles correlated well with the presence of mild dystonic signs. However, the analysis of brainstem interneuronal excitability did not enable recognition of those patients with head tremor who could potentially develop cervical dystonia. The enhancement of brainstem interneuronal excitability found in approximately 30% of patients with head tremor could be related to plastic changes triggered by increased activity of the cranial muscles.

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Purpose:The purpose of this study was to evaluate the incidence and outcome of surgically treated superior oblique palsy (SOP) and the factors involved in its resolution.Methods:We performed a retrospective study of 76 patients who underwent surgery for SOP. We recorded data from the physical examination and the number and type of procedures performed. Favorable outcome was defined as resolution of or improvement in torticollis (≤5°) and diplopia in primary position (PP) and downgaze or as vertical deviation (VD) <5 prism diopters (pd) in PP and 10 pd in the oblique diagnostic position.Results:Mean age was 33.12 years. Congenital SOP was the most frequent type (65.8%). Mean preoperative VD was 15.89 ± 9.94 pd, decreasing to 3.07 ± 4.36 pd after surgery. Associated horizontal deviation was recorded in 51.32% of cases. The mean number of procedures was 1.37 ± 0.62 (range 1–4), with 69.7% of patients requiring only one procedure. The mean number of muscles operated on was 1.96 ± 1.01 (inferior oblique being the most frequent). A greater reduction in VD after surgery was observed in patients with congenital SOP (P = 0.04). Although none of the factors evaluated influenced surgical outcome, amblyopic patients had a greater risk of reoperation (P = 0.04). A favorable outcome was achieved in 75% of cases. Mean follow-up was 37.08 months.Conclusion:Congenital SOP was twice as frequent as acquired SOP and although surgery was successful in most cases, a greater reduction in VD was obtained in congenital cases. Amblyopia was identified as a risk factor for reoperation.

We present a 68-year-old patient with essential tremor who was treated with propranolol hydrochloride (80 mg daily) and gabapentin (900 mg daily) after a history of mild success of gabapentin alone in relieving his symptoms. The patient had several daily episodes of paroxysmal dystonic movements in both hands. After reducing the propranolol dose to 40 mg daily, the dystonic movements resolved. This case suggests a synergistic effect between propranolol and gabapentin.