Dan Boghen

The treatment of the restless legs syndrome with clonazepam: a prospective controlled study

The effect of clonazepam on the restless legs syndrome was studied in a group of 6 patients. Following a drug-free period, 3 patients received clonazepam for 4 weeks followed by placebo for 4 weeks thereafter and 3 patients received the same medication and for the same length of time but in reverse order. The effectiveness of the medication was evaluated by means of a self-rating system in which patients assigned a score daily to the degree of discomfort experienced in the previous 24 hours. Three patients improved on clonazepam but 2 of these also improved on placebo. Clonazepam was not shown to be significantly more effective than placebo in the treatment of RLS.

Apraxia of lid opening A review

Apraxia of lid opening (ALO) is a disabling syndrome characterized by an inability to open the eyes at will. It is frequently found in association with blepharospasm (BSP), with which it may be readily confused. ALO has been called by a variety of names and attributed to a number of different mechanisms. The syndrome is not widely recognized and has been the subject of few articles; there has been no major review. Herein are reviewed the arguments surrounding its name, and its clinical features, pathophysiology, and treatment in light of the current medical literature are discussed. Goldstein and Cogan’ have used the term apraxia of lid opening for the nonparalytic abnormality characterized by “difficulty in initiating the act of lid elevation” in four patients with basal ganglia disease. Earlier, Schilde? described a difficulty of eye opening in two patients who also had difficulty in closing their eyes, and Riese3 reported ALO in a patient with a frontotemporal injury. One of the patients reported by Schilder had Huntington’s chorea, a disease also present in one of Goldstein and Cogan’s patients. Subsequent reports emphasized the presence of the syndrome in extrapyramidal diseases such as multiple system a t r ~ p h y , ~ Parkinson’s d i ~ e a s e , ~ ~ MPTP intoxi~at ion,~ and in particular, progressive supranuclear palsy (PSP).4,7Jo-14 Difficulty or inability to open the eyes on command consistent with ALO was previously reported in seven patients during the acute stage of strokes involving the nondominant hemi~phere.’~-*~ In five of these patients, lid function recovered within l month of onset. ALO is also associated with acanthocyto~is,’~ Wilson’s disease,20 and motor neuron disease.21 BoghenZ2 first drew attention to the association between ALO and BSP. The occurrence of ALO in BSP was 7% in one study7 and 10% in another.23 Tolosa et al.24 found ALO in 12 of 22 consecutive patients with BSP. The majority of reported cases of ALO occur in association with BSP. ALO can also occur in isolation.7,25-27 Apraxia of lid opening: the term. The suitability of the term ALO was challenged by Lepore and Duvo i~ in .~ To them, the presence of extrapyramidal disease in their patients disqualified the syndrome as an apraxia. This objection is countered by the syndrome’s occurrence in the absence of basal ganglia disease and the fact that its neuroanatomic basis and pathophysiology are unknown. A number of different designations, none of which is a truly adequate substitute, are suggested as being more appropriate than ALO. One of these, focal eyelid d y ~ t o n i a , ~ is nonspecific and is based on presumed evidence that the syndrome is dystonic in nature. Others, such as involuntary levator palpebrae inhibition (ILPI),4 blepharocolysis (eyelid inhibition),28 pretarsal b l epharo~pasrn ,~~ and atypical blepharospasmz6 are based on the mistaken assumption that the syndrome is due to a single mechanism. Freezingz9 and akinesia of lid function30 are terms used in relation to Parkinson’s disease and were proposed before researchers realized that the majority of the patients lack extrapyramidal signs such as akinesia and rigidity. Moreover, although “freezing” is a vivid descriptive term, other qualifications such as “in the closed position,” are required to describe the phenomenon adequately. Despite that when applied to the syndrome under consideration “apraxia” doesn’t have its customary connotation of a dissociation between language interpretive areas and the motor cortex, ALO is probably still the most useful designation for this syndrome. As others have n~ ted , l~ -~O it is brief, clear, and in common use, and addresses the main feature of the syndrome, namely the inability to open the eyes at will with preservation of the ability to open them and to keep them open at other times. “ALO” is consistent with the common meaning of the term “apraxia,” which is used when a patient “cannot perform a motor action to command despite both an adequate understanding of the action and the elementary ability to carry it As is the case for

Effectiveness of Botulinum Toxin in the Treatment of Spasmodic Torticollis

Thirty-two patients with spasmodic torticollis were assessed quantitatively for posture deformity, tremor and range of neck movement, and qualitatively for pain and global subjective disability. All patients were then treated with intramuscular botulinum toxin injections into appropriate neck muscles. Fifty-three treatments were administered using dosages of toxin in the range of 50-100 U per muscle. The maximum dose administered at a single sitting was 280 U. The progress of the patients was assessed during an 18-month period. Seventy-five percent of patients showed documented improvement in both subjective and objective parameters and were considered treatment successes. Pain improved in 65%, posture in 65%, tremor in 50% and range in 46%. The side effects that occurred were transient and included fatigue, dysphagia, neck weakness, hoarseness and local pain. This study demonstrates that treatment with botulinum toxin is of significant benefit for the majority of patients with spasmodic torticollis.

Botulinum toxin improves lid opening delays in blepharospasm-associated apraxia of lid opening.

Lid movement and EMG of the orbicularis oculi (OOc) were analyzed in 10 patients with apraxia of lid opening associated with blepharospasm before and after botulinum toxin treatment. The latencies to onset and to complete the eye opening and the time during which eye opening was sustained were studied in relation to OOc activity and compared with control values obtained in 12 healthy subjects. Following treatment there was an improvement of all lid opening measurements, a decrease of the abnormally prolonged OOc activity, and a reduction of the functional disability.

Electromyographic studies of congenital mirror movements

We studied two patients with congenital mirror movements. In one, the movements were associated with impaired sensation and a cervicodorsal meningocele. In the other, no abnormality of the nervous system was found. In an EMG study, the normal temporal characteristics, response latency, duration, and amount of EMG on the normal and mirror sides suggest that similar motor commands are responsible for both voluntary and mirror movements.

Prolonged orbicularis oculi activity A major factor in apraxia of lid opening

Background: Apraxia of lid opening (ALO) is a nonparalytic inability to open the eyes at will in the absence of visible contraction of the orbicularis oculi (OOc) muscle. Objective: To test the hypothesis that sustained activity of the OOc is a major factor in the difficulty in opening the eyes in this condition. Methods: Lid movement detected in an electromagnetic field and electromyogram activity of the septal and pretarsal portions of the OOc were recorded in 12 healthy control subjects and 12 patients with a clinical diagnosis of ALO. The latencies to onset and to complete eye opening and the time during which eye opening was sustained were measured and analyzed in relation to OOc activity. Results: The lid opening latencies and the lid movement duration were significantly increased in patients compared with control values. An abnormal persistence of OOc activity was present in 10 of the 11 patients with a delay in complete lid opening. The complete lid opening delay showed a strong positive correlation with the time it took to inhibit the OOc activity. This relationship fit the same linear regression in both groups. Conclusions: Patients with ALO show significant delays in eye opening. An abnormal persistence of OOc activity, detectable electromyographically but not clinically, could be the main factor contributing to the delay in lid opening in these patients.

Botulinum Toxin Therapy for Apraxia of Lid Opening

DAN BOGHEN,a,c VIORIKA TOZLOVANU,b,c ANDREEA IANCU,b,c AND ROBERT FORGETb,c,d aNeuro-ophthalmology Section, Centre Hospitalier de l’Université de Montréal (Hôtel-Dieu), Montréal, Québec, Canada bCentre de Recherche Interdisciplinaire en Réadaptation du Montréal Métropolitain, Institut de Réadaptation de Montréal, Montréal, Québec, Canada cCentre de Recherche en Sciences Neurologiques, Faculté de Médecine, Université de Montréal, Montréal, Québec, Canada dÉcole de Réadaptation, Université de Montréal, Montréal, Québec, Canada

Visual function improvement in patients with macroprolactinomas treated with bromocriptine

We studied eight patients who had visual field defects secondary to prolactin-secreting macroadenomas and who had improved visual function with bromocriptine treatment without surgery or radiation. We recommend bromocriptine as a primary treatment for prolactin-secreting macroadenomas. If therapy is effective, continued regular neuro-ophthalmologic, endocrine, and imaging studies are necessary, because treatment with bromocriptine must be continued indefinitely.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

Background: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well. Methods: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits. Results: Schwannomas of the third, fourth, and fifth nerves resulted in fluctuating deficits of all 3 ocular motor nerves. Persistent nodular cranial nerve enhancement was present on sequential MRI studies. Several episodes of transient oculomotor (III) deficts were associated with headaches, mimicking ophthalmoplegic migraine. Conclusions: Cranial nerve schwannomas may result in relapsing and remitting cranial nerve symptoms.

Cerebral Microbleed Causing an Acute Stroke-like Episode in a CADASIL Patient.

Cerebral microbleeds (CMBs), visualized on gradient-recalled echo (GRE) T2 or susceptibility-weighted imaging magnetic resonance imaging (MRI) sequences, and thought to reflect minor leakage from fragile arterial micro-vessels, are classically considered to be clinically silent. They are frequent incidental findings in MRI scans of healthy subjects, especially in the elderly population and even more prevalent in patients with acute ischemic and hemorrhagic stroke. However, increasing evidence has called into question the asymptomatic nature of CMBs. In particular, they appear to be associated with an increased risk of incident ischemic and hemorrhagic strokes as well as recurrent strokes. Greater controversy surrounds the possibility that CMBs can cause acute stroke-like episodes. In fact, CMBs are often associated with a degree of surrounding tissue necrosis; however, in theory, the brain tissue damaged by an acutely developing CMB is too small to produce focal neurological symptoms. Nevertheless, a few case reports of acute focal deficits with negative diffusionweighted imaging (DWI) brain-MRI sequences seem to suggest that a strategically located CMB could, indeed, cause an acute stroke syndrome. These few published reports have invariably described patients with classical risk factors for small vessel disease.