Speech-induced changes in corticospinal excitability

The aim of the experiments was to investigate the effects of speech on the excitability of corticospinal pathways to human hand muscles. Single transcranial magnetic stimuli were given randomly over the hand area of either the left or right motor cortex of 10 right-handed and 3 left-handed normal volunteers. Electromyographic responses were recorded in the relaxed first dorsal interosseous muscle while the subjects (a) read aloud a piece of text, (b) read silently, (c) spoke spontaneously, or (d) made sounds without speaking. The only consistent effect across subjects occurred during task a, which significantly increased the size of responses evoked in the dominant hand of all subjects, but had either no effect (8 subjects) or a smaller effect in the nondominant hand. Tasks b and d had no reliabl effects, whereas task c tended to increase response size in both hands. Control measurements suggest that the effects in task a were caused by changes in cortical rather than spinal excitability. This is the first demonstration of lateralized speech effects on the excitability of cortical arm areas. The results provide a useful adjunct to other tests of cerebral dominance, using only single- rather than repetitive-pulse cortical stimulation.     

Bell's palsy-induced blepharospasm

We report two patients with blepharospasm that appeared during the recovery phase of Bell's palsy. It is well known that hemifacial spasm occasionally appears after Bell's palsy; however, blepharospasm associated with Bell's palsy has been rarely reported so far. Blepharospasm appeared within a month after the onset of Bell's palsy, suggesting that a certain causal relationship may be present between Bell's palsy and blepharospasm. We speculate that corneal irritation caused by lagophthalmos contributes to the induction of blepharospasm. Another speculation is that abnormal afferent input from the paralyzed side contributes to the abnormal sensitization of the blink reflex, thereby facilitating the induction of abnormal facial motor outputs such as blepharospasm. Received: 17 May 2001, Accepted: 27 August 2001     

Stretch reflex blepharospasm

We studied a patient with blepharospasm provoked by stretching the orbicularis oculi muscles. With the eyes closed, EMG of the orbicularis oculi at rest revealed spontaneous rhythmic muscle activity that was not visible. When she tried to open her eyes, repetitive jerking blepharoclonus and tonic blepharospasm rendered her functionally blind. Passive stretching of the orbicularis oculi evoked a burst of EMG activity with two components. Touch or pinprick did not elicit such reflex activity. Anesthesia of the supraorbital nerves abolished muscle responses to stretch and improved the blepharospasm.     

Speech-induced primary lingual dystonia: a rare focal dystonia

Lingual dystonia, a type of focal dystonia that may be primary or secondary, is related to brain damage, neuroleptic use, neurodegenerative, metabolic, and neurodevelopmental disorders, varicella infection, and so on. However, primary lingual dystonia induced by speaking is a rare type of focal dystonia that is usually idiopathic in origin and is characterized by increased tonus of the tongue, which causes protrusion only during speaking. This report describes a 55-year-old male patient with lingual dystonia during speech. One interesting clinical feature of this case was that the speech disturbance improved while the patient vocalized a praise-like hymn in a manner that resembled singing.     

Blinking and essential blepharospasm

Blepharospasm consists of a series of repeated involuntary contractions of the orbicularis oculi and may occur alone, with other dystonias, or as a symptom of an underlying disorder. This paper describes the clinical picture of blepharospasm, and then theories of aetiology and approaches to treatment are discussed. Four illustrative case histories are presented. Present evidence supports an organic cause. Treatment is difficult, but recent developments allow a rational approach.     

Dystonic frowning without blepharospasm

We report on two cases who presented with involuntary facial grimacing and frowning. Blepharospasm has never been documented in one, and the other case showed difficulty in lid opening only in the early phase of the disease. Botulinum toxin was effective for both. This is a hitherto unreported presentation of upper facial dystonia without interference of eyelid motor control.     

Speech-induced aphasic seizures in epilepsy caused by LGI1 mutation

Summary:  Purpose: Patients with autosomal dominant lateral temporal lobe epilepsy (ADTLE) may have seizures precipitated by sound or speech. We have examined a patient with speech-induced seizures caused by an LGI1 mutation (C46R).
Methods: A clinical study and a video-EEG recording using interrogative speech as the activation procedure was performed in a 23-year-old man.
Results: He had experienced short episodes of sensory aphasia in situations in which he was suddenly verbally addressed. Voices became distorted, and he could not comprehend despite hearing words. The day after a late party, his girlfriend unexpectedly spoke to him. Her speech became unintelligible to him. He did not reply and had a generalized tonic–clonic (GTC) seizure. During an EEG, he was suddenly asked for the names of his siblings. He answered, but lost understanding of the further conversation and described how syllables floated together with an echoing character. With a versive movement to the right, another GTC occurred. In the EEG, rhythmic 6-Hz activity built up in the frontotemporal areas starting on the left side with bilateral and posterior spreading. Postictal slowing was symmetrical, and no aphasia was noted on awakening.
Conclusions: To our knowledge, this is the first video-EEG recorded seizure in LGI1 -caused ADTLE. This peculiar seizure semiology and precipitating effect of speech may serve as a marker for identifying further individuals with this particular phenotype and genotype and may indicate that the LGI1 gene may have a physiologic function connected to the human capacity for speech and language.     

Cognitive disturbances in primary blepharospasm

The common belief that primary dystonia is a purely motor disorder with no anatomical substrate and no other accompanying neurological dysfunction has recently been challenged. In addition, there is increasing evidence that the basal ganglia besides motor control, plays a role in cognitive functioning. However, no systematic cognitive performance evaluation has been carried out in patients with primary blepharospasm (BS), one of the most common forms of adult dystonia. We evaluated a series of 20 patients with primary BS and a group of 17 controls matched by severity of mood symptoms, age, and sex. BS patients performed significantly worse on the Luria sequencing test, Purdue pegboard test, reciprocal coordination, tactile denomination, and reverse visuospatial span and the differences persisted after correction for age, duration of disease, severity of BS, and degree of depression. The Wisconsin card sorting test showed no statistical difference, but BS patients made more errors and more perseverative answers than expected according to population means, whereas the control group performed poorly but within normal parameters. Our findings suggest broad cortical involvement in focal dystonia that is not correlated with the severity or duration of dystonia. © 2009 Movement Disorder Society     

Auditory brain-stem responses in blepharospasm

The auditory brain-stem response (ABR) has been reported to detect abnormalities in both the auditory pathways and in adjacent structures. Ten of 35 consecutive patients with blepharospasm were found to have abnormal ABRs involving poor form and delayed peak latency of positive components III or V. Abnormal ABRs in approximately 30% of patients with essential blepharospasm suggest pathology in the brain-stem of a substantial proportion of patients with this form of cranial-cervical dystonia.     

Epidemiology of primary blepharospasm.

We review epidemiological data on primary blepharospasm (BSP). There is a large variation in the stated prevalence of BSP, with crude estimates ranging from 16 to 133 per million in different studies. A large proportion of this variability may be the result of differences in physician education on BSP. Age and female gender may increase the risk of developing BSP. The few case-control studies focusing on adult dystonias including BSP showed an increased risk in association with family history of dystonia and/or postural tremor, prior head and face trauma, and prior eye disease (e.g., blepharitis and keratoconjunctivitis), and a decreased risk associated with cigarette smoking. No association was found with age-related medical conditions such as hypertension and diabetes, family history of parkinsonism, and a history of anxiety or depression. Broocks et al. [Am J Psychiatry, 1998;155:555-557] found a significantly higher frequency of obsessive-compulsive symptoms in BSP than hemifacial spasm despite the clinical similarity. Among putative risk factors for BSP, age at onset, female gender, and prior head or face trauma may affect spread of dystonia to adjacent body regions. While limited, the body of epidemiological data support the idea that environmental and familial, possibly genetic, factors may both be important in the etiology of BSP.     

Improvement of blepharospasm with Zolpidem.

Zolpidem (ZLP) is an imidazopyridine that binds to GABA receptors. We report on improvement of blepharospasm in 3 patients treated with ZLP. The GABAergic action of this drug on the output structures of the basal ganglia could explain the improvement of blepharospasm in these patients.     

Excessive daytime sleepiness in idiopathic blepharospasm

ObjectiveTo explore the frequency of excessive daytime sleepiness (EDS), and its impact on quality of life and its associated clinical factors in idiopathic blepharospasm.MethodsThis cross-sectional study was carried out in 425 idiopathic blepharospasm patients and a group of 424 age-matched and sex-matched healthy subjects. EDS was assessed with the Epworth Sleepiness Scale (ESS) in all subjects. Other clinical characteristics of patients with idiopathic blepharospasm including motor symptoms, sleep quality, depression, anxiety, cognition, and quality of life were also assessed.ResultsEDS was significantly more frequent in patients with idiopathic blepharospasm than in controls (22.1% vs 12.3%; p < 0.05). Blepharospasm patients with EDS scored significantly higher in Jankovic Rating scale, Hamilton Rating Scale for Depression (HDRS), Hamilton Rating Scale for Anxiety (HARS), and significantly lower in Montreal Cognitive Assessment (MoCA) and 36-Item Short Form Health Survey (SF-36) than those without EDS (p < 0.05). The binary logistic regression model indicated that male, younger age of onset of blepharospasm, higher motor scores, higher HDRS scores, and lower MoCA scores were associated with the presence of EDS in patients with blepharospasm (p < 0.05).ConclusionsRecognition and management of EDS in idiopathic blepharospasm patients is necessary as the occurrence of EDS was associated with higher motor burden, more serious mood and cognitive disturbances, and poorer quality of life. Our results suggest that blepharospasm may exhibit abnormal sleep-wake patterns and further support the clinical heterogeneity of the disease.