Majid Aramideh

Medisch Centrum Alkmaar | MCA · Department of Neurology

Objective: Trials for additional or alternative treatments for cervical dystonia (CD) are scarce since the introduction of botulinum neurotoxin (BoNT). We performed the first trial to investigate whether dystonic jerks/tremor in patients with CD respond to the selective serotonin reuptake inhibitor (SSRI) escitalopram. Methods: In a randomised,...

Centralization of intravenous thrombolysis (IVT) for acute ischemic stroke in high-volume centers is believed to improve the door-to-needle times (DNT), but limited data support this assumption. We examined the association between DNT and IVT volume in a large Dutch province. We identified consecutive patients treated with IVT between January 2009...

Background: Studies of genetic association between TOR1A and adult-onset primary torsion dystonia have contradictory results. Methods: The authors genotyped TOR1A single nucleotide polymorphisms rs1801968, rs2296793, rs1182 and rs3842225 in a cohort of clinically well characterized cervical dystonia patients (n=367) and constructed haplotypes. T...

Background and purpose: The purpose of this study was to determine the incidence of adult cerebral venous thrombosis. Methods: A retrospective cross-sectional study was conducted among all 19 hospitals located in 2 Dutch provinces serving 3.1 million people. Adult cerebral venous thrombosis cases diagnosed between January 1, 2008, and December 3...

Cervical dystonia, a late onset focal dystonia, has a complex genetic background. Multiple lines of evidence point to a role for aberrant dopamine levels in dystonia. We assessed whether common variation within genes that regulate brain dopamine levels and in key genes of the dopamine metabolic pathway, modulate the risk for cervical dystonia. DNA...

The focal primary torsion dystonias (FPTDs) form a group of clinical heterogeneous syndromes and can be considered a genetic complex disease; it is thought to be primed by genetic variants with variable impact and triggered by non-genetic factors. Thorough clinical description of FPTDs cohorts is sparse but essential for further progress in genetic...

Mutations in THAP1, a gene encoding a nuclear pro-apoptotic protein, have been associated with DYT6 dystonia. First reports on the phenotype of DYT6 dystonia show an early onset dystonia with predominant cranio-cervical and laryngeal involvement. Here we assessed the frequency and phenotype of THAP1 mutation carriers in a large Dutch cohort of adul...

A 71-year-old woman presented with recurrent episodes of headache accompanied by hemihypoesthesia, fever, aphasia, reduced consciousness and worsening of pre-existing ataxia. Brain imaging revealed atrophy of the cerebellum. The white cell count in the cerebrospinal fluid was slightly increased. The patient had a family history of migraine and cere...

In three patients, a man aged 46 years and two women aged 74 and 38 years, respectively, restless legs syndrome (RLS) was diagnosed. The second patient had a low serum ferritin concentration. The patients were successfully treated with ropirinol, iron suppletion and gabapentine, and pramipexol, respectively. With a prevalence of 7% in the general p...

A 34-year-old alcoholic man had neurological and cardiac symptoms. The patient was admitted to the hospital for acute painful sensory disturbances and severe weakness of the feet. Neurological and electrophysiological investigation revealed axonal sensorimotor polyneuropathy that was most prominent in the legs. Cardiac assessment showed signs and s...

This chapter discusses electrophysiological assessments of patients with the so-called “cranial hyperkinetic syndromes.” The term cranial hyperkinetic syndrome is introduced to indicate those disorders that are characterized by involuntary contractions of the muscles innervated by the cranial nerves. As it is impossible to discuss all types of cran...

Recording of the brainstem reflexes enable to assess the functional integrity of these reflexes within the brainstem and their afferent and efferent connections. Many structural abnormalities, such as tumors or infarctions at the level of the brainstem may abolish reflexes. Recording of different brainstem reflexes may enable to exclude structural...

The kinematics and neurophysiological aspects of eyelid movements were examined during spontaneous, voluntary, air puff, and electrically induced blinking in healthy human subjects, using the direct magnetic search coil technique simultaneously with electromyographic recording of the orbicularis oculi muscles (OO-EMG). For OO-EMG recordings, surfac...

One of the major complications of a peripheral facial nerve palsy (PFP) is the occurrence of corneal ulceration due to lagophthalmos—that is, incomplete closure of the affected eyelid. It is widely accepted that lagophthalmos after PFP is directly caused by paresis of the orbicularis oculi muscle (eyelid closure muscle). Yet, some of the signs and...

An overview is provided on the physiological aspects of the brainstem reflexes as they can be examined by use of clinically applicable neurophysiological tests. Brainstem reflex studies provide important information about the afferent and efferent pathways and are excellent physiological tools for the assessment of cranial nerve nuclei and the func...

Implications: In this case report we describe a technique used to provide local analgesia for surgical procedures. Although this technique has a reduced risk of complications, we present a patient who experienced a life-threatening paralysis without loss of consciousness during an attempted brachial plexus block with a posterior approach.

Patients with facial nerve palsy are at risk of developing corneal ulceration because of lagophthalmos (incomplete closure of the affected eyelid). Lagophthalmos could result from thixotropy of the levator palpebrae muscle--that is, the formation of tight crossbridges between the actin and myosin filaments of the muscle fibres causing stiffness of...

Patients with facial nerve palsy are at risk of developing corneal ulceration because of lagophthalmos (incomplete closure of the affected eyelid). Lagophthalmos could result from thixotropy of the levator palpebrae muscle—that is, the formation of tight crossbridges between the actin and myosin filaments of the muscle fibres causing stiffness of t...

Keywords:blink;blink reflex;eyelid movement;eye movement;brainstem

©2002 BMJ Publishing Group

To investigate possible abnormalities of the blink reflex pathways, we analyzed the latencies and amplitudes of the blink reflex responses in the orbicularis oculi (Ooculi) muscle, following supraorbital nerve stimulation, in 19 patients with blepharospasm, 16 patients with torticollis spasmodica and 22 control subjects. Furthermore, in order to ex...

We compared various electrodiagnostical tests in patients with hemifacial spasm and in patients who developed synkinesia after Bell's palsy. We examined the evoked blink reflexes in the orbicularis oculi (o. oculi) and orbicularis oris (o. oris) muscles in 23 patients with hemifacial spasm (HFS), in 10 patients with synkinesia after Bell's palsy (B...

The neural relationships between eyelid movements and eye movements during spontaneous, voluntary, and reflex blinking in a group of healthy subjects were examined. Electromyographic (EMG) recording of the orbicularis oculi (OO) muscles was performed using surface electrodes. Concurrently, horizontal and vertical eye positions were recorded by mean...

Previous studies have shown that clinical localization of trigeminal nerve lesions is inaccurate as compared with MR imaging findings. The purpose of our study was to ascertain the added value of electromyographic (EMG) investigation of the trigeminal nerve reflexes for the improvement of lesion localization and for the preselection of patients for...

Studies of blinks have revealed the reciprocal relationship between the innervation patterns of the levator palpebrae superioris (LP) and the orbicularis oculi (00) muscles (Gordon 1951; Björk and Kugelberg 1953; Becker and Fuchs 1988; Evinger 1991; Aramideh et al. 1994a) resulting in a downward movement of the upper eyelid. Immediately prior to a...

R1 and R2 blink reflex responses to single and paired stimuli were investigated in 23 control subjects: 21 patients with blepharospasm (BSP), 20 patients with torticollis spasmodica (TS), and 23 with hemifacial spasm (HFS). For paired stimuli, we compared measurements of area and peak responses at two and three times R2 threshold. R1 and R2 indices...

The value of physical examination in detecting involved neck muscles in cervical dystonia (CD) is uncertain and little is known about changes in electromyographic (EMG) features after botulinum toxin type A (BTA) treatment. In a double-blind, randomized study we recorded the EMG activities of 420 neck muscles in 42 patients with CD before and after...

Functionally and anatomically, the orbicularis oculi (OO) muscle can be subdivided in a pretarsal, a preseptal, and an orbital portion. In the rhesus monkey, fluorescent and neuronal retrograde tracing experiments were performed in the pretarsal or the orbital portion of the OO muscle, or both, using fast blue, diamidino yellow, and wheat germ aggl...

Unlabelled: There is little agreement on which outcome measures to use to express the efficacy of treatments for cervical dystonia. We analyzed change scores on various scales of 64 new patients with cervical dystonia before and after repeated injections with botulinum toxin. Method: The association between change in impairment (Tsui), and chang...

We investigated the long-term effect of botulinum toxin type A (BTA) on impairment as well as functional health in terms of disability, handicap, and quality of life in 64 patients with cervical dystonia. These patients, who first participated in a double-blind trial, were followed for another 12 months. Fifty-four patients continued treatment afte...

We investigated the long-term effect of botulinum toxin type A (BTA) on impairment as well as functional health in terms of disability, handicap, and quality of life in 64 patients with cervical dystonia. These patients, who first participated in a double-blind trial, were followed for another 12 months. Fifty-four patients continued treatment afte...

We report on a blink reflex abnormality observed in two patients, which provides additional information on the central pathways mediating this reflex. Autopsy was performed in one patient and MRI in the other. In the first patient there was a small lesion at the dorsal middle third of the lateral tegmental field and in the second patient at the lev...

We report on a blink reflex abnormality observed in two patients, which provides additional information on the central pathways mediating this reflex. Autopsy was performed in one patient and MRI in the other. In the first patient there was a small lesion at the dorsal middle third of the lateral tegmental field and in the second patient at the lev...

In the cynomolgus monkey, motoneurons innervating the levator palpebrae superioris muscle form a nucleus within the oculomotor nuclei called the central caudal nucleus. After double fluorescent neuronal retrograde tracing experiments, using fast blue and diamidino yellow as tracers in the levator palpebrae superior muscles, labelled motoneurons (30...

We reviewed 21 studies (8 blinded and 13 open) on the treatment of botulinum toxin type A for cervical dystonia, directed to the health aspects used to evaluate the patients' response to treatment (Medline search 1985-1993, English language literature). The prerequisite for comparing the treatment results was that studies had to evaluate similar as...

Two patients, men aged 82 and 53 years, with weakness of the facial muscles after surgery in the posterior fossa had keratitis caused by the inability to blink and to close the eye. Botulinum toxin type A was injected into the levator palpebrae superioris muscle under electromyographic control. A selective, reversible paralysis of this muscle was i...

We describe three patients with a late presentation of congenital muscular torticollis to emphasize that this non-dystonic type of torticollis has to be considered in the differential diagnosis of cervical dystonia, even in adult patients. Surgery is the treatment of choice. Postoperatively, two of our patients had a transient lesion of the accesso...

A patient with neurofibromatosis type I and breast carcinoma developed a bilateral but asymmetric blepharospasm and paresis of the left abducens muscle over a 2-week course. MRI disclosed a small lesion in the left dorsomedial lower pontine region. Electrodiagnostic investigations revealed bilateral R1 responses after stimulation of the left suprao...

Purpose: In blepharospasm involuntary eyelid closure may be accompanied by abnormal eye movements. To obtain better understanding in underlying neuropathological mechanisms we examined the relationship between blinks and eye movements in a group of normal controls. Methods: During blinking, eye movements were evaluated at various gaze angles. Refle...

Sixty patients with idiopathic cervical dystonia were treated a total of 240 times with botulinum toxin type A (BTA). Selected muscles were injected with BTA under electromyographic (EMG) guidance. The clinical effect was measured on the Tsui scale and a 10-point anchored visual analogue scale. A dosage of 150-300 mouse units was used in 77% of the...

The response to botulinum toxin type A was compared after two injection techniques in 45 patients with blepharospasm. Initially, patients were treated according to a triple injection technique; two injections into the upper eyelid and one injection into the lower eyelid. Subsequently, without altering the dose, the same patient group received two f...

Electromyographic (EMG) activity of orbicularis oculi and levator palpebrae muscles was recorded to study the origin of involuntary eyelid closure in 33 patients. The evoked blink reflex in all patients and in 23 controls was also studied. To examine the excitability of facial motoneurons and bulbar interneurons in individual patients and to compar...

Assessment of the functional status in patients with blepharospasm is of major importance for clinical practice and outcome studies. The Blepharospasm Disability Scale (BDS) is specifically directed to measure the disability in these patients. The metric properties of this instrument were evaluated. Reliability, validity, and responsiveness to with...

We describe clinical and EMG findings in three patients with an inability to reopen the eyes after voluntary closure of the eyelids. Synchronous EMG recording from the levator palpebrae (LP) and orbicularis oculi (OrbOc) muscles revealed that after voluntary closure of the eyelids and upon the command to open the eyes, all three patients were unabl...

We report on four patients with involuntary eyelid closure and eye movement disorders. Three were healthy until the onset of their illness and one had a mild generalized choreoathetosis and dystonia due to kernicterus. Electromyographic recording revealed solely blepharospasm in two patients and blepharospasm in combination with involuntary levator...

We report on five patients with involuntary eyelid closure, diagnosed as blepharospasm and referred to use for treatment with botulinum A toxin. Synchronous electromyographic (EMG) recording was performed from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles. In the first two cases, EMG registration showed alternating, s...

Electromyographic (EMG) recording was performed synchronously from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles in 28 patients referred to us for treatment of blepharospasm with botulinum A toxin. At the time of this study, 19 patients were under the treatment with botulinum, four started treatment shortly after the...

We report on four patients with involuntary eyelid closure and eye movement disorders. Three were healthy until the onset of their illness and one had a mild generalized choreoathetosis and dystonia due to kernicterus. Electromyographic recording revealed solely blepharospasm in two patients and blepharospasm in combination with involuntary levator...

Discussion of clinical symptoms and differential diagnosis of blepharospasm and treatment with botulinum A toxin. Blepharospasm is an involuntary spasmodic contraction of the eyelids. Within a few years 35%-70% of the patients becomes severely disabled. Prospective, open study. Academical Medical Centre, Amsterdam. In the period 1985-1992 we have s...

Clinical and neurophysiological examinations were performed on seven patients with hereditary spastic paraplegia and on eight patients with primary lateral sclerosis. The results were compared with those obtained from a group of 39 control subjects. Prolonged latency times and decreased amplitudes of the posterior tibial nerve (PTN) somatosensory e...

Thirty-nine patients from six families with hereditary motor and sensory neuropathy type I and control subjects were included in this study. A neurological deficit score (NDS) was derived from a neurological examination and compared with neurophysiological test findings. Further, sensory nerve conduction velocities (SNCV) were compared with the mot...