Prediction of disease progression in Miller Fisher and overlap syndromes.

Patients with Miller Fisher syndrome (MFS) may have a relatively mild clinical course or progress to Guillain-Barré syndrome (GBS) with limb weakness (MFS-GBS overlap syndrome). Other variants in this spectrum are GBS with ophthalmoparesis and Bickerstaff's Brainstem encephalitis (BBE). To compare the clinical course of MFS and overlap syndromes and to identify predictors of disease progression. In a prospective study of 170 patients with GBS and variant forms, 37 (22%) had a MFS, MFS-GBS overlap syndrome, ophthalmoplegic GBS or BBE. The clinical, serological, and electrophysiological features were compared. Twenty-three patients presented with MFS, of which 10 (43%) developed limb weakness (MFS-GBS overlap syndrome). All these transitions occurred in the first week after onset of symptoms. There were no differences in the clinical, electrophysiological and serological features at entry between MFS and MFS-GBS. Twelve patients had ophthalmoplegic GBS and the disease severity at nadir and outcome was worse than in the patients with a MFS-GBS overlap syndrome. No early predictors for progression from MFS to MFS-GBS overlap syndrome were found. All transitions occurred in the first week. This finding implicates that all patients with MFS need careful monitoring for at least 1 week.

Ophthalmoplegic "migraine" or recurrent ophthalmoplegic cranial neuropathy: new cases and a systematic review.

Ophthalmoplegic migraine is a poorly understood neurologic syndrome characterized by recurrent bouts of head pain and ophthalmoplegia. By reviewing cases presenting to our centers in whom the phenotype has been carefully dissected, and systematically reviewing all published cases of ophthalmoplegic migraine in the magnetic resonance imaging (MRI) era, this review sets out to clearly define the syndrome and discuss possible etiologies. We found that in up to one-third of patients, the headache was not migrainous or associated with migrainous symptoms. In three-quarters of the cases involving the third nerve, there was focal nerve thickening and contrast enhancement on MRI. Observational data suggest systemic corticosteroids may be beneficial acutely. The etiology remains unclear, but may involve recurrent bouts of demyelination of the oculomotor nerve. "Ophthalmoplegic migraine" is a misnomer in that it is probably not a variant of migraine but rather a recurrent cranial neuralgia. A more appropriate name might be "ophthalmoplegic cranial neuropathy."

Meningitis following relapsing painful ophthalmoplegia in aspergillus sphenoidal sinusitis: a case report.

We report the case of a 58-year-old woman in whom relapsing painful ophthalmoplegia related to a mycetoma of the sphenoid sinus gave origin to meningitis with markedly depressed glucose levels in the cerebrospinal fluid. Surgical exeresis of the mycetoma allowed aetiological diagnosis (aspergillosis) and--together with antimycotic therapy--led to durable clinical response.

Electrophysiological study of four patients with Fisher syndrome: a mechanism of areflexia.

Electrophysiological studies were performed on four patients with the Fisher syndrome (two 4-year-old boys, a 5-year-old boy and a 9-year-old girl). Motor nerve conduction velocity (MNCV), nerve action potential, F wave, H reflex and T reflex were measured at the stage when areflexia was present but ataxia and ophthalmoplegia had recovered. MNCV and SNCV by compound mixed nerve potential were normal in all our patients. The amplitude of the muscle responses (M-responses) and compound mixed nerve action potentials were also within normal limits. The latencies of the F waves and F wave conduction velocities were normal. However, H-reflexes and T-reflexes were absent in all four cases. Our results suggested that areflexia in the Fisher syndrome is attributed to the desynchronization of the impulses or the partial conduction block of GIa fibers.

High CSF lactate and pyruvate content in Kearns-Sayre syndrome.

We studied lactate and pyruvate concentrations in CSF and blood of a patient with Kearns-Sayre syndrome (KSS), 3 patients with ocular myopathy and 11 normal control subjects. We found significant elevation of lactate and pyruvate in the CSF of the patient with KSS, suggesting a disorder of CNS lactate-pyruvate metabolism.

Migraine, Tolosa-Hunt syndrome and pleocytosis. Correlation or coincidence?

A critical analysis is presented of the problem whether the migraine attack causes CSF-pleocytosis or vice versa, on the basis of a series of 3 cases, including one of Tolosa-Hunt syndrome due to sarcoidosis, the other two ultimately showing chronic leptomeningitis. The evidence leads to the conclusion that migraine does not cause pleocytosis and pleocytosis does not cause migraine: both are correlated by an underlying common denominator.

CSF myelin basic protein levels in acute optic neuritis and multiple sclerosis.

Normal CSF-MBP levels as determined by a RIA were less than 6.2 ng/ml CSF (mean 3.9). Eighty percent of patients with acute optic neuritis have CSF-MBP levels greater than 6.2 ng/ml (mean 7.6 ng/ml CSF). Five of 7 patients with acute internuclear ophthalmoplegia due to an initial exacerbation of demyelination have CSF-MBP levels above 6.2 ng/ml (mean 6.8 ng/ml). Fifty percent of MS patients with chronic progressive disease have CSF-MBP levels above 6.2 ng/ml (mean 6.7 ng/ml). MS patients experiencing monosymptomatic exacerbations show elevated CSF-MBP levels in 75% in 75% of cases (mean 8.2 ng/ml). MS patients experiencing polysymptomatic exacerbations show significantly higher levels of CSF-MBP (mean 22.3 ng/ml) than the patients with monosymptomatic exacerbations. Ninety-five percent of MS patients experiencing polysymptomatic exacerbations have elevated levels of CSF-MBP.

Multiple mononeuritis and radiculitis with erythema, pain, elevated CSF protein and pleocytosis (Bannwarth's syndrome).

Four patients with Bannwarth's syndrome were examined. One to three weeks weeks after the appearance of a large erythematous skin lesion they developed pain of variable localisation. Four days to four weeks later uni- or bilateral facial palsies and other nerve lesions developed. CSF showed elevated protein and mononuclear pleocytosis. There was a defect of the blood-brain barrier and increased intrathecal production of IgG. Facial nerve latencies were increased 4 to 7 times in three cases and normal in one case examined 15 months after onset. Conduction velocity along other nerves was normal apart from slight slowing in one diabetic patient. Recovery was incomplete in three patients 8 to 15 months after onset of disease and complete in one patient after 15 months.

[Syndrome of ophthalmoplegia, ataxia and areflexia of Fisher in a 14 year old boy (author's transl)]. / Fisher-Syndrom (Ophthalmoplegie, Ataxie, Areflexie) bei einem 14jährigen Knaben.

The syndrome of ophthalmoplegia, ataxia and areflexia first mentioned in 1932 by Collier and in 1938 by van Bogaert was described more precisely in 1956 by Fisher. It represents a specific clinical entity and is considered to be a variant of idiopathic polyradiculoneuropathy (Guillain-Barré). The most striking features are external (sometimes also internal) ophthalmoplegia, hyporeflexia or areflexia and cerebellar ataxia. During the first days paresthesias are frequently present whereas other disturbances of sensibility are not common. Additional signs of neurological lesions may be observed in association with the syndrome. Spinal fluid shows the typical albumino-cytological dissociation. The disease is characterized by its benign course. The etiology remains unclear, therapy is not necessary.

Fisher syndrome in childhood.

Three children, age 2, 7, and 12, developed Fisher syndrome: inability to walk because of ataxia, complete areflexia, and ophthalmoplegia. Ptosis was prominent in all the children, but the pupillary response to light was affected only in one child. Limb weakness was never present, and sensation normal. One patient was obtunded for several days. Two had prodromal upper respiratory tract illnesses, and the third patient was bitten by an insect 2 days before her symptoms began. Cerebrospinal fluid (CSF) protein content was moderately increased in all patients, but only one child's CSF had a pleocytosis. CSF gamma globulin levels were normal. In the acute phase, F waves and H responses were absent in two cases but returned to normal with clinical recovery. All three patients had marked electroencephalographic (EEG) abnormalities that later improved. The EEG pattern suggested a brainstem disorder. These findings plus the obtundation, gaze paralysis, and ataxia indicate that in Fisher syndrome there is parenchymal involvement of the central nervous system with or without nerve root involvement.

The tolosa-Hunt syndrome: further clinical and pathogenetic considerations based on the study of eight cases.

The Tolosa-Hunt syndrome (THS) is characterized by remittent and sometimes recurring episodes of painful ophthalmoplegia. The etiopathogenesis is still unclear and is an object of controversy. A non-specific granulomatous process of the wall of the cavernous sinus is claimed by many authors as the possible cause, on the basis of a few pathological studies. Other authors suggest the possible role of autoimmune or specific inflammatory processes localized in the retroorbital perineural tissues. The clinical, laboratory and radiological findings of the eight cases reported in the agreement with those previously described in the literature. However, the visual evoked potentials (VEP) were delayed in three of the four cases in which they had been studied. This finding, together with the observation that some analogies exist between THS and other well known neuritic processes of the cranial nerves, may suggest that at least in some cases THS may be related to an ocular polyneuritis.

[Progressive extrinsic ophthalmoplegia; case report]. / Oftalmoplegia extrínseca progressiva; registro de um caso.

A case of progressive ophtalmoplegia and ptosis affecting a 12 year old boy is reported. A miopatic pattern was detected by the electromyography, the cerebrospinal fluid showed an increase of the protein content and retinal pigmentary degeneration was detected. This findings call the attention for a more widespread degenerative disorders affecting not only the muscular sistem but other organs as well.

[Progressive External Ophthalmoplegia (authors transl)]. / Die progressive externe Ophthalmoplegie.

The case of a 16-year-old boy with progressive external ophthalmoplegia ("Ophthalmoplegia plus") is reported. Clinical symptoms consisted of complete external ophthalmoplegia, ptosis, short stature, atrophy and weakness of skeletal muscles, and elevated csf protein. Abnormal mitochondria were found by electron microscopy in scattered atrophic fibres of the muscle biopsy. The syndrome and it's relevance for paediatric differential diagnosis is discussed.

[Neuroophthalmological complications through liquor leakage after surgical operation on the spinal canal for diagnostic i.e. therapeutic purposes (author's transl)]. / Neuroophthalmologische Komplikationen durch Liquor=leckage nach diagnostischen bzw. therapeutischen Eingriffen am Spinalkanal.

The rare liquor leakage syndrome with cranial nerve lesion, which preferably affects the eye-muscle nerves, can be caused either by further leakage of liquor cerebrospinalis through the dura hole made by the lumbar puncture - or after a surgical operation on the spinal canal leaving a dura closure, which is not tight. This does not occur after a suboccipital puncture. A report is given on three cases with different eye-muscle paresis after a simple diagnostic lumbar puncture, after a lumbosacral myelography and after a resection of the sacral nerve roots including the filum terminale and caudal dural sac. The prognosis of the eye-muscle paresis is good. Restitutio ad integrum resulted. A fourth case with temporary loss of sight of one eye is reported after a lumbar air encephalography, but the eye nerves were damaged before as a result of previous tuberculous basal meningitis. Only partial recovery of the visual power occurred.