1/116. Monocular involuntary eyelid closure. OBJECTIVES: Monocular blepharospasm (MBLS) is precipitated by ipsilateral ocular pathology and becomes bilateral over time. Two patients are reported with monocular visual disturbance exhibiting monocular involuntary eyelid closure (MIEC), resembling MBLS. MATERIAL AND methods: A 31-year-old female with hyperthyroidism developed monocular polyopia with left eye vision on binocular fixation followed by ipsilateral visual extinction. Monocular vision was otherwise normal. A 57-year-old male with renal failure developed monocular blindness secondary to retinal hemorrhage. RESULTS: The first patient had anisocoria and proptosis. brain magnetic resonance (MR) was normal. The second patient had involuntary movements of the left eye. Head computerized tomography (CT) was normal. Both patients exhibited MIEC of the eye with visual impairment that could be overcome voluntarily, but reappeared upon distraction. Neither of the two developed BLS in long-term follow-up. CONCLUSION: MIEC represents a complication of ipsilateral ocular pathology in the absence of other involuntary movements, apraxia of eyelid opening, or blepharospasm (BLS). MIEC needs to be distinguished from MBLS, since not all MBLS becomes bilateral. ( info) |
2/116. blepharospasm and apraxia of eyelid opening in lithium intoxication. We present the case of a 72-year-old woman with a history of a bipolar mood disorder chronically treated with lithium. Upon having the dose increased, she developed an acute confusional state accompanied by blepharospasm (BS) and apraxia of eyelid opening. gait instability with frequent falls, pyramid tract signs, and postural tremor in both hands were also evident. On withdrawing lithium, symptoms remitted within 2 weeks. This patient illustrates that BS and apraxia of eyelid opening may be triggered by lithium overdose. Our case warrants the inclusion of lithium in the list of drugs liable to induce such movement disorders. ( info) |
3/116. Lamotrigine-induced blepharospasm. movement disorders such as tremor and ataxia occur commonly during therapy with antiepileptic drugs (AEDs). Dystonias, however, are rare. blepharospasm, although reported with neuroleptic agents, has never been reported with AEDs. Our patient developed blepharospasm during therapy with lamotrigine. ( info) |
4/116. Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature. The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved. ( info) |
| We report a patient initially diagnosed as having ocular myasthenia gravis who showed progressive ophthalmoparesis and pseudoblepharospasm together with positive acetylcholine receptor antibodies. Repeated evaluation with high-frequency repetitive stimulation revealed an incremental response and elevated titers of antibodies against presynaptic calcium channels, confirming lambert-eaton myasthenic syndrome. Systemic evaluation revealed no malignant neoplasm but revealed euthyroid Hashimoto's disease. Immunomodulative therapy including plasma exchange and administration of an immunosuppressent (azathioprine) combined with a potassium-channel blocker (3,4-diaminopyridine) reduced the ocular abnormalities. We conclude that the ocular manifestations in this patient were probably caused by lambert-eaton myasthenic syndrome. ( info) |
6/116. clozapine in the treatment of neuroleptic-induced blepharospasm: a report of 4 cases. BACKGROUND: blepharospasm, the forcible closure of eyelids, is an infrequent consequence of neuroleptic treatment that, when severe, can interfere with the ability to walk, drive, or work. Like tardive dyskinesia, blepharospasm can be disfiguring and aesthetically distressing, contributing to the increased stigmatization of patients. case reports: We report 4 patients with DSM-IV schizoaffective disorder, paranoid schizophrenia, or chronic undifferentiated schizophrenia who developed neuroleptic-induced blepharospasm. In all patients, blepharospasm remitted without the reemergence of psychosis within 3 to 5 months of treatment with clozapine, 100-200 mg/day. CONCLUSION: The results suggest that clozapine may successfully treat neuroleptic-induced blepharospasm without the reemergence of psychosis in patients with schizophrenia, schizoaffective disorder, or schizophreniform disorder. ( info) |
7/116. A Yorkshire family with adult-onset cranio-cervical primary torsion dystonia. Although a family history is described in approximately 20% of patients, large families with adult-onset craniocervical primary (idiopathic) torsion dystonia (PTD) are rare. We report a new British family with cranio-cervical dystonia. Seventeen members of the family were examined. Five cases were diagnosed as definite PTD and one as probable PTD. Mean age at onset was 29 years (range, 19-40 yrs). The phenotype was characterized by adult-onset cranio-cervical dystonia in all affected cases. A few cases had additional voice tremor and/or postural arm tremor. The GAG deletion in the DYT1 gene was excluded in the index case. Linkage analysis was performed between the disease and several marker loci spanning DYT6 and DYT7 regions, and haplotypes were reconstructed in all subjects. Although linkage analysis was not completely informative, reconstructed haplotypes excluded linkage between the disease and either DYT6 or DYT7. This report confirms that familial cranio-cervical dystonia is genetically heterogeneous, and further studies of other PTD families with similar clinical features are needed to identify other new genes. ( info) |
8/116. Superselective neurectomy with periorbital primary reconstruction for blepharospasm. Case report. A 66-year old man with blepharospasm and ptosis of the brow was treated with a combined procedure in which the branches of the facial nerve were excised at the margin of the orbicularis, and the periorbital area was reconstructed simultaneously. The condition improved after the treatment with no occurrence of either oral complications or facial anaesthesia. ( info) |
| Gangliogliomas of the lateral ventricle are unusual tumours of the central nervous system. We report a 25-year-old female who presented with a 4-week history of generalized headaches associated with left-sided blepharospasm. Clinical examination revealed bilateral papilloedema but no focal neurological deficits. Computed tomography and magnetic resonance imaging demonstrated a large lesion within the left lateral ventricle. Gross total surgical resection of the lesion was performed through a para-sagittal, trans-callosal approach. Histopathological analysis revealed the diagnosis of ganglioglioma. The post-operative course was uneventful, with complete resolution of both the headaches and blepharospasm. The presentation with an ipsilateral blepharospasm has never previously been reported in the setting of a lateral ventricular tumour. ( info) |
| OBJECTIVE: Blepharoclonus (BLC) denotes a large amplitude, involuntary tremors of the orbicularis oculi muscles, observed during gentle closure of the eyelids. BLC may follow major head trauma. Four patients with arnold-chiari malformation (ACM) and BLC are described. MATERIALS AND methods: The first patient had facial numbness for 5 months; the remaining patients had headaches following minor head or cervical spinal injuries. brain magnetic resonance imaging (MRI), electroencephalogram (EEG) blink reflexes, mental and facial nerve responses and facial electromyogram (EMG) were performed. RESULTS: All patients exhibited ACM on brain MRI. The first patient had coincidental dural venous malformation, empty-sella turcica and familial digital dysplasia. She exhibited oculopterygoid synkinesis. The last 3 patients had posttraumatic headache; the second and third patients had limited features of ehlers-danlos syndrome (EDS). The second patient had cervical spinal fusion and the fourth a cervical syrinx. All the patients had BLC on gentle eyelid closure. CONCLUSION: BLC is an underdiagnosed neuro-ophthalmological sign of ACM. ( info) |