Risk factors for spread of primary adult onset blepharospasm: a multicentre investigation of the Italian movement disorders study group.

Objectives: Little is known about factors influencing the spread of blepharospasm to other body parts. An investigation was carried out to deterrmine whether demographic features (sex, age at blepharospasm onset), putative risk, or protective factors for blepharospasm (family history of dystonia or tremor, previous head or face trauma with loss of consciousness, ocular diseases, and cigarette smoking), age related diseases (diabetes, hypertension), edentulousness, and neck or trunk trauma preceding the onset of blepharospasm could distinguish patients with blepharospasm who had spread of dystonia from those who did not.

Methods: 159 outpatients presenting initially with blepharospasm were selected in 16 Italian Institutions.

Possible risk factors for primary adult onset dystonia: a case-control investigation by the Italian Movement Disorders Study Group.

Objectives: Little is known about the aetiology of idiopathic adult onset dystonia. The Italian Movement Disorders Study Group promoted a case-control study on some hypothetical risk factors including past medical events, life events, life habits, occupational hazards, and family history of dystonia, parkinsonism, and tremor.

Methods: Cases affected by idiopathic adult onset dystonia (age at symptom onset >20 years, duration of disease >one year and View Article and Find Full Text PDF

Case report: sequential bilateral carotid dissection leading to Raeder's syndrome.

A 39-year-old man complained of unilateral headache, diplopia and marked anterior neck oppression "like a necklace". MRA was used to study this case of subsequent bilateral internal artery dissection (ICAD) which initially appeared as Raeder's syndrome. MRA proved to be a non-invasive alternative for studying stenosis, occlusions and dissections of the intracranial and extracranial vessels.

Major neurological sequelae of lumbar epidural anesthesia. Report of three cases.

We here report the major permanent neurological complications that developed in three patients after epidural anesthesia. MR clearly showed that paraplegia, which arose one and nine days after anesthesia, was due to epi-subdural haematoma in the first case and epidural abscess in the second. The sudden left lower limb palsy in the third patient was caused by a paracentral ischemic lesion all along the conus-epiconus following a probable trauma of the cord during the insertion of the needle.

Abnormal projection of corticospinal tracts in a patient with congenital mirror movements.

A 31 year-old woman with familial congenital mirror movements not associated with other neurological defects underwent a detailed neurophysiological evaluation including: voluntary electromyographic activity recorded from upper limbs in response to acoustic stimuli, motor evoked potentials from the thenar muscles to focal transcranial magnetic stimulation, F waves from upper extremities, scalp somatosensory evoked potentials and long-latency responses from thenar muscles to electric stimulation of the median nerve. The results were consistent with the presence of fast-conducting pathways connecting each hand motor cortex with both contra- and ipsilateral spinal motoneurones.

A further case of giant intrasellar carotid aneurysm mimicking a pituitary adenoma: the relevance of a multivariate approach in differential diagnosis.

We describe a 53 year old woman who progressively showed a left extrinsic oculomotor paresis, at first suggesting ocular myasthenia, which was ruled out by a negative Tensilon test. A CT scan of the brain revealed a pituitary mass consistent with an adenoma which was confirmed by endocrinological investigation and standard MRI. Carotid angiography, performed the day before the scheduled trans-sphenoidal surgery, clearly showed a giant intrasellar aneurysm of the left carotid syphon.

"Epilepsia partialis continua" due to multifocal encephalitis: favourable outcome after immunoglobulin treatment.

The case of a young woman with EPCK is described in which neoplastic and vascular disorders were excluded. Supported by EEG and PET, EPCK was imputed to multifocal encephalitis notwithstanding serological and CSF negativity. Cerebral biopsy confirmed the inflammatory nature of the affection, although the etiologic agent was not identified.

Localization of the pathological process in Miller Fisher syndrome.

A 64 year old woman died at the third attack of MFS. Histological examination demonstrated segmental demyelination and axonal swelling of the peripheral nerves studied, oculomotor included. In the C.

Isolated amnesia following a bilateral paramedian thalamic infarct. Possible etiologic role of a whiplash injury.

A previously healthy 45 years old carpenter suffered a whiplash injury in a road accident on July, 18th, 1990. He continued to work in spite of occipital headache, episodic sweatening and slight hypersomnia. On August, 8th, 1990 while parking his car into the deck of a ferry-boat he was found slightly confuse and markedly amnestic.

Benign focal amyotrophy: a longitudinal study (13-15 years) in 3 cases.

We report three young patients, two male and one female, with B.F.A.

[Occasional disclosure of large arachnoid cysts in 2 subjects].

Two old men in whom the CT and MR scans had unexpectedly shown an enormous arachnoid cyst in the fronto-temporo-parietal region, underwent neuropsychological examination. This did not show any neuropsychological abnormality. This is in accordance with the hypothesis that the cyst represents a dysontogenetic disorder with very slow growth, leaving functionally normal the cerebral structures shifted.

Responsiveness of idiopathic spasmodic torticollis to botulinum A toxin injection. A critical evaluation of five cases.

Five patients with Idiopathic Spasmodic Torticollis (IST), one associated with Meige's disease, have been cured by local injection of purified botulinum "A" toxin (BT). The therapeutic effect on the different subjects treated proved to be variable, and often the effect varied as well on the same subject from session to session. The response was only partly related to the dose of BT injected, and appeared inversely proportional to the number of hyperactive muscles.

Total mesencephalic "locked-in" syndrome. A case report and review of the literature.

A 63-year-old man suddenly fell in a state of apparent coma. An electroencephalogram (EEG) performed at a local hospital revealed the maintenance of a reactive alpha rhythm over the posterior regions of the scalp, allowing a diagnosis of alert coma. The persistence of minimal degrees of alertness and ability to communicate made the clinical picture classifiable as a "locked-in" syndrome (LIS).

Use of botulinum toxin in Meige's disease.

Four patients with severe Meige's disease (blepharospasm-oromandibular dystonia) have been treated, after having given an informed consent, by local injections of purified botulinum toxin type "A". Previous systemic therapy with anticholinergics, dopamine antagonists and other drugs had been unsuccessful in all these subjects. Each patient was treated by saline solution injected with the same method as botulinum toxin, just once.

Beneficial effect of botulinum A toxin in blepharospasm: 16 months' experience with 16 cases.

After introducing the problem of blepharospasm, we report our experience on treatment with purified botulinum A toxin in 16 cases of blepharospasm, symptomatic in two and essential in 14, than had not responded to drugs. The changes in intensity and frequency of spasm after treatment were evaluated on a clinical scale and by review of videotapes. The beneficial effect appeared within a week in most patients, lasting from 6 to 28 weeks (mean 13), and reached the maximum at the third-seventh week.

Acute and subacute akinetic-rigid parkinsonian syndrome mimicking a meningoencephalitis.

Four cases of acute and subacute akinetic-rigid parkinsonian syndrome are described. The clinical picture initially simulated a meningoencephalitis, which was escluded by the negative CSF findings and the aspecific electroencephalogram. In the first case the parkinsonism was attributable, also on the basis of the autoptic findings, to the prolonged neuroleptic therapy; in the other cases a toxic-infectious condition, through a damage on liver function, revealed a subclinical latent parkinsonian syndrome.

Tolosa-Hunt syndrome versus recurrent cranial neuropathy. Report of two cases with a prolonged follow-up.

Two patients are described who had suffered for 12 years from episodes of painful ophthalmoplegia consistent with a Tolosa-Hunt syndrome (THS) alternating with palsies of cranial nerves other than the oculomotor (fifth motor and seventh on both sides). These two cases, as well as other similar ones previously reported in the literature, suggest that THS may sometimes be a variant of so-called recurrent cranial neuropathy, which is a benign and poorly understood clinical entity on an inflammatory or ischaemic basis.

Acquired idiopathic sensory neuropathy. Report of three cases with a prolonged follow-up.

We have followed for more than ten years three women suffering from a S.N. which appeared in their fourth decade.

Peripheral ophthalmoplegia as the only sign of late-onset fibrous dysplasia of the skull.

A 49-year-old woman presented with a left eye abduction-elevation defect and a bilateral internal rectus palsy of peripheral origin. Thyroid function, cranial computed tomographic scan, and cerebrospinal fluid examination were normal, as were the prostigmine test for myasthenia gravis and the guanidine hydrochloride test for myasthenic syndrome. Skull radiography showed osteosclerotic and osteolytic areas, with slight orbital distortion, and there was hyperactivity in an isotopic scan.

CT signs relevant to the diagnosis of tuberculous meningitis: contrast uptake at tentorial level.

A marked rim of contrast uptake in the subarachnoid spaces at the level of the tentorium on CT scanning was a bit of a riddle. When correctly interpreted, the finding, in agreement with the clinical and CSF findings, led to the diagnosis of tuberculous meningitis in a 72 year old woman. The case is reported to further the correct assessment of the CT image in this disease.

Total extrinsic ophthalmoplegia as only paraneoplastic sign two years before X-ray diagnosis of bronchial carcinoma.

An extrinsic total ophthalmoplegia developing two years before radiologic evidence of bronchial carcinoma and onset of Eaton-Lambert myasthenic syndrome is reported. Clinical and ENG data showed the neuromuscular location of the ophthalmoplegia, but repeated Tensilon and Prostigmine tests were negative. CT scan and CSF examinations revealed neither carcinomatous metastases nor inflammatory CNS disease.

Unusual association of Eales disease with multifocal neurological deficit.

A man who had suffered from Eales disease at the age of 20 presented an acute Brown-Séquard spinal syndrome when he was 30, followed shortly after by paralysis of the right abducens and recurrent laryngeal nerves. Steroid treatment apparently relieved the myelopathy while the neurological deficits have remained unchanged for the past 14 years. In view of the clinical and CSF findings the neurological deficits are attributed to the same disease process as that underlying the peripheral retinal abnormalities characteristic of Eales disease rather than to multiple sclerosis.

Possible effectiveness of plasmapheresis on the neurological complications in a case of acute intermittent porphyria.

A 56 year old woman with acute intermittent porphyria presented mainly motor polyneuropathy, ophthalmoparesis of probable supranuclear origin and a mild organic brain syndrome. The neurological complications gradually cleared during a course of plasmapheresis. The fairly rapid improvement could conceivably have been fortuitous but it may well have been due to plasmapheresis.

[Late myelopathy caused by ionizing radiation. Considerations on 3 cases].

Three cases of tardive myelopathy were identified among more than one thousand patients treated for tumors by X-rays at the Institute of Radiology of the University of Florence during the period 1974-83. In two of these, the clinical picture was that of a partial Brown-Séquard syndrome while in the third case it corresponded to a transverse myelitis. The myelopathy of the former patients can be attributed to the total radiation dose, which was very close to the tolerance limit.

[Cranial injury disclosing an asymptomatic arachnoid cyst of the sylvian fossa].

In a 21 year-old man suffering from a post-traumatic neurotic syndrome, the cranial CT scan revealed an arachnoid cyst in the left Sylvian fissure, which had been completely asymptomatic before. The surgical emptying of the cyst modified neither the CT finding nor the neurotic syndrome. The latter disappeared once the insurance controversy was resolved.