Adult brainstem glioma presenting with isolated persistent hemifacial spasm or facial nerve palsy

ObjectAdult brainstem gliomas are a rare group of heterogeneous brain tumors. Classical clinical presentation includes progressive impairment of cranial nerves associated with long tract signs. The prognosis and response to treatment are poor; nevertheless, some patients do have a long survival. The objective of this study was to describe a series of patients with an isolated persistent hemifacial spasm and/or facial nerve palsy as the presenting symptom of a brainstem glioma.MethodsFourteen patients from 3 French hospitals (Paris, Caen, Lille) were included. Clinical and radiological features and overall survival were retrospectively analyzed. A review of the literature of similar cases was performed.ResultsMean age at diagnosis was 35 years (range 19–57 years). Mean duration of facial nerve involvement before diagnosis was 17 months (range 1–48 months). Tumors were characterized on MRI by a lateralized location in the pons, a T1-weighted hyposignal, a T2-weighted hypersignal and no contrast enhancement after Gadolinium injection except for 2 cases. Biopsies were performed in 10 cases and showed 8 low-grade and 2 high-grade gliomas. All the patients were initially treated with radiotherapy and 6 patients with chemotherapy after progression. Eleven patients died from tumor progression. Median survival time was 90 months.ConclusionsAdult brainstem gliomas revealed by a progressive isolated involvement of the facial nerve seem to have particular clinico-radiological features of slow progressive tumors and may be associated with long patient survival.     

The electromyogram in facial myokymia and hemifacial spasm

Electromyographic findings in 5 patients with facial myokymia due to multiple sclerosis and in 10 patients with hemifacial spasm are described and compared.Many of the spontaneously discharging motor units in myokymia are not under voluntary control.In hemifacial spasm the high frequencies of discharge reached in blink bursts are similar to those recorded during normal involuntary blinking.The possible sites of origin of the discharges are briefly discussed.     

The association of hemifacial spasm and facial pain.

The recognition of an association between trigeminal neuralgia and ipsilateral hemifacial spasm has been delayed by confusion over the nomenclature of the two conditions. Three patients are presented who had facial pain associated with hemifacial spasm. The findings on investigation of these patients, and an analysis of the literature, suggests that the combination is almost inevitably associated with pathological processes in the posterior fossa, particularly anomalous, ectatic or aneurysmal blood vessels.     

Electrodiagnostic studies of the facial nerve in peripheral facial palsy and hemifacial spasm

Electrodiagnostic (EDX) assessment is one of the most important aspects in the evaluation of the two most common disorders of the facial nerve: facial palsy and hemifacial spasm. Facial palsy is usually an acute disorder that resolves in a few weeks but, in a number of cases, leads to a postparalytic facial syndrome featuring muscle synkinesis, myokymia, and involuntary mass contractions of muscles on the affected side. Hemifacial spasm is usually a chronic disorder characterized by paroxysms of involuntary, clonic, and synchronous twitching of all facial muscles on the affected side. EDX studies provide information on lesion location and severity, pathophysiology underlying the two disorders, and differential diagnosis between syndromes presenting with abnormal facial muscle activity. This monograph is intended to describe the most relevant EDX findings in the two disorders and the most appropriate timing for the examinations in order to provide useful information for prognosis and therapeutic decision-making.     

微血管减压与面神经梳理治疗面肌痉挛

目的探讨治疗面肌痉挛的有效方法。方法对42例面肌痉挛病人行微血管减压加面神经梳理术。结果术后痉挛立即停止者37例,逐渐停止者5例。结论微血管减压加面神经梳理术是治疗面肌痉挛的有效方法,即使术中未发现明确的责任血管。对术后复发的病例,只要正确处理,二次手术仍有效。     

Reorganization of sensorimotor gating after peripheral facial palsy starts at brainstem*

Background and objective We aimed to analyze the alterations in sensorimotor gating at brainstem after peripheral facial palsy (PFP). To examine sensorimotor gating, we used prepulse modulation (PPM) of blink reflex (BR). We also recorded BR recovery to identify excitability changes in the facial nucleus.

Patients and method We included 33 patients and 39 recordings. Control group was composed of 16 healthy subjects. Simultaneous bilateral baseline BR, BR recovery at ISI of 300-ms and BR-PPM at ISI of 100-ms recordings were performed after stimulation of trigeminal nerve on right sides of healthy subjects and on both sides of patients. Severity of PFP and time lapse from the onset was noted.

Results Mean R1 amplitude was increased, whereas mean R2 and R2c magnitude were reduced in all groups after prepulse stimulation. However, multivariate ANOVA showed significance at group level (patients and healthy subjects), at prepulse level (no prepulse and 100-ms prepulse) and group and prepulse level. Suppression of R2 or R2c was lower on both sides of patients compared to healthy subjects and the deficit first started on the symptomatic side.

Conclusion Suppression of R2 and R2c after prepulse stimulation is reduced in PFP suggesting decreased filtering of facial sensory input at brainstem level. Trigeminal sensitization at brainstem develops early after PFP.     

Behind the facial twitch: depressive symptoms in hemifacial spasm

BACKGROUND: Depression impairs psychosocial and occupational functioning and contributes to significant morbidity and mortality. Hemifacial spasm (HFS) causes social embarrassment and visual and verbal disability. OBJECTIVE: We examined; (1) the prevalence and predictive factors of depressive symptoms (Becks Depression Inventory (BDI) and clinical assessment) in HFS and (2) the sensitivity and specificity of BDI as a screening and diagnostic tool in HFS. METHODS: A large cohort of HFS patients in a movement disorders clinic was clinically evaluated and the BDI self-administered by patients. Univariate analysis and multivariate logistic regression were undertaken to investigate the effect of age, gender, body-mass index, duration and severity of HFS on the outcome of BDI score. ROC (receiver operating characteristics) analysis was utilized to evaluate the sensitivity and specificity and discriminative property of the scale. RESULTS: There were 90 HFS patients with a mean age of 54.4+11.1 (35-79) years, comprising of 58.9% women and with a mean severity HFS score of 2.9+0.8 (range 1-4). The mean BDI score was higher in depressed HFS than in non-depressed HFS (19.7+6.7 vs 4.2+4.9, p<0.0001). Female gender and a younger age were risk factors (p=0.07). In the multivariate analysis, the severity of HFS was an independent predictor of BDI scores (p<0.0001). The AUC was 97.1% suggesting excellent discriminative property of BDI. For cut-off score of 12/13, the sensitivity was 93.3%, specificity 94.7%, Positive Predictive Value 77.8% and Negative Predictive Value 98.6%. CONCLUSIONS: The prevalence of depressive disorder in HFS was 16.7%, with younger women at greater risk. The severity of HFS was positively correlated with the severity of depressive symptoms. The BDI can be a complimentary screening and/or diagnostic instrument for depressive disorder in HFS. Early diagnosis of at-risk patients will prevent unnecessary morbidity and mortality.     

面肌痉挛显微血管减压术后迟发性面瘫

目的 探讨面肌痉挛显微血管减压术(MVD)后迟发性面瘫的病因、治疗及预后.方法 随访我院同一个术者自2007年1月至2009年11月行MVD治疗的415例面肌痉挛患者中术后出现迟发性面瘫者,研究面瘫的发生时间、持续时间及预后情况.结果 415例患者中20例术后出现迟发性面瘫,发病率4.8%.面瘫发生时间为术后5～14 d(平均10.7 d),持续时间为14～150 d(平均49.7 d);不论是否经过治疗,所有出现迟发性面瘫的患者均能够临床治愈.结论 MVD术后迟发性面瘫并不少见,发病原因尚不明确,可能与疱疹病毒感染有关,具有临床自愈性,预后佳.     

Microvascular decompression of the facial nerve for hemifacial spasm in youth

OBJECTS: The goal of this study was to investigate the differences between clinical findings in youth and in adulthood on microvascular decompression (MVD) of the facial nerve for the treatment of hemifacial spasm (HFS). METHODS: We retrospectively evaluated 855 patients who underwent MVD from January 1985 to July 1999. In our series of 33 young HFS patients, all patients had definite offending vessels. Interestingly, pathologic tortuous vertebral artery as a possible etiology was more rarely observed in young HFS patients (1/33 patients, 3.0%) than in adult patients (61/822 patients, 7.4%) (P < 0.05). We did not observe any atomical variations of the vessels or any arachnoidal thickening around the root entry zone and cerebellopontine cistern in youths. Furthermore, young HFS patients did not necessarily have poorer surgical outcomes than adult HFS patients. CONCLUSIONS: Our results suggest that the cause and progress of HFS are the same in youth as in adulthood, even though the pathogenesis of early onset remains unclear.     

Magnetic stimulation in hemifacial spasm and post–facial palsy synkinesis

The facial nerve was stimulated trascranially with a magnetic stimulator in 14 normal controls, 14 hemifacial spasm patients, and 16 post–facialpalsy synkinesis patients. Magnetic stimulation in normal controls revealed muscle responses which had latencies with a mean value of 4.99 ± 0.49 ms and amplitudes of 2.41 ± 1.08 mV. In the same group, transosseal conduction time was calculated to be 1.20 ± 0.13 ms. In the hemifacial spasm group, the amplitudes of the responses on the affected sides were lower as compared to the unaffected sides (mean values 1.78 vs. 2.41 mV, P = 0.01). Also, the threshold to magnetic stimulation was elevated on the affected sides. These findings are suggestive of the presence of a hypoexcitability to magnetic stimulation in the root entry zone. In the post–facial-palsy synkinesis patients, magnetic stimulation of the affected sides resulted in responses with long latencies and low amplitudes (mean latency 6.34 ms, mean amplitude 0.90 mV). In the recordings made with magnetic stimulation, the difference of the latencies between the two sides was larger as compared to those obtained by electrical stimulation. The transosseal conduction time was also remarkably prolonged on the affected side. These findings may suggest that magnetic stimulation can be an effective method of showing intracranially located lesions of the facial nerve. © 1993 John Wiley & Sons, Inc.     

Baclofen in hemifacial spasm

A 58-year-old woman with long-standing hemifacial spasm experienced dramatic relief of symptomatology while receiving baclofen (Lioresal) in a daily dosage of 37.5 mg. Clinical response was noted within 48 h and she remained asymptomatic during 12 months of treatment. No toxic or side-effects were observed. Controlled studies are needed to establish the role of baclofen in hemifacial spasm.     

Baclofen in hemifacial spasm

Six patients with idiopathic hemifacial spasm refractory to the usually employed medications were successfully treated with baclofen. Adverse side effects were found in only 1 patient (memory loss) which was controlled with administration of piracetam. We suggest that there is an association between stress and certain cases of hemifacial spasm and that such stress related cases may respond positively to baclofen.