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Total, bilateral ophthalmoplegia is very rare. More than 50% of cases are Miller Fisher (MFS) and Guillain-Barré (GBS) syndromes. There is a correlation of MFS with anti-GQ1b antibodies. High levels of GQ1b gangliosides are found in myelin sheathes of cranial nerves supplying the extraocular muscles. This may explain the association of anti-GQ1b antibodies with ophthalmoplegia. Anti-GQ1b were also found in cases of GBS accompanied by ophthalmoplegia, atypical MFS (MFS without ataxia), MFS/GBS overlap syndromes and Bickerstaff brainstem encephalitis. This has led some authors to classify them as 'anti-GQ1b syndromes'. In this article we describe a diagnostically difficult case of a patient with a very rare, total bilateral paralysis of all ocular muscles, accompanied by bilateral ptosis, diminished tendon reflexes of upper extremities, paresis and hypoesthesia of the left upper extremity.

Guillain-Barré syndrome (GBS) is the most common acquired polyradiculoneuropathy in childhood. It has been conventionally regarded as a disease exclusively of the peripheral nervous system. The involvement of the central nervous system (CNS) in patients with GBS has been rarely described. The purpose of the study was to delineate the extent of subclinical neurological changes of CNS in children and adolescents with this syndrome. Thirty patients aged 8-18 years with GBS were treated in the Chair and Department of Developmental Neurology. The upper and lower limbs paresis dominated in clinical pictures of these patients. Cranial nerve involvement was noticed in 9 children. An ascending bulbar paralysis was noted in four cases. Sensory symptoms were found in all children. The nerve conduction velocities were abnormal in all examined patients with GBS. The control group for neurophysiological studies consisted of 66 healthy children and adolescents at the matched age. Evoked potentials (EP) were registered by means of Multiliner (Toennies, Germany). Brainstem evoked potentials (BAEP) and visual evoked potentials (VEP) were always performed in the same conditions with the same equipment for the children and adolescents in the GBS and control group, according to the guidelines of the International Federation of Clinical Neurophysiology (IFCN). The following BAEP parameters were considered: the latencies of waves I to V, interpeak latencies I-III, III-V and I-V. The latencies of N75, P100, N145 as well as the amplitudes of N75/P100, P100/N145 for mono- and binocular stimulations of VEP were analysed. BAEP showed abnormalities in 6 cases and VEP in 5 cases. The statistically significant prolongation of the latencies of waves III, V and interpeak I-V were found in children and adolescents with GBS. The BAEP changes may reflect impairment at the brainstem level in these patients. In VEP recording in GBS the prolongation of latencies P100 and N145 without changes in amplitudes were found. These parameters were statistically significant in comparison to the control group. VEP results suggest the involvement of visual pathway in examined children and adolescents. EP can be used as a complementary method for the evaluation of clinically silent lesion in the auditory and optical tracts in GBS.

Adrenoleukodystrophy (ALD) is a hereditary disease related to abnormalities in the structure and function of peroxisomes. The nature of disorder arises from defective process of beta-oxidation of very-long-chain fatty acids and their accumulation in various organs. ALD may present as a few phenotypes urologic symptomatology of which depends on proportions of the brain, spinal cord and peripheral nerves affection. Below, we present a case (and its family) of a cerebral type of adrenomyeloneuropathy--a rare form of adrenoleukodystrophy, and discuss its clinical manifestation as well as biochemical, hormonal, electrophysiological, neuropsychological and magnetic resonance imaging (MRI) diagnostics.

The paper presented the significance of EEG activation in diagnosing epilepsy in children and adolescents. EEG records of 108 patients between 3-19 years of age with the occurrence of various types of paroxysmal disorders who were admitted to Chair and Department of Developmental Neurology University of Medical Sciences in Poznań were analysed. Standard EEG was administered to all children twice: the first examination at rest with hyperventilation (HV) and photostimulation (FS) and the second one after total or partial sleep deprivation (DS). At awaking state normal records were stated in 57% of cases, in 15% paroxysmal changes in HV or FS appeared, in the rest of cases (28%) generalised paroxysmal disorders, localised changes, lateralised or diffused were noted. Among EEG activation methods DS occurred to be the most effective after it, 52 patients (48%) were diagnosed for generalised paroxysmal changes, 20% for localised, 3% lateralised and for 6% for diffused changes. Normal EEG were recorded in 22% of examined children and adolescents. The epilepsy were diagnosed in 59 (55%) of patients mostly with generalised seizures (28) partial secondarily generalised (16) and partially complex (11). In 49 (45%) children were diagnosed with other paroxysmal disorders.

68 patients with Guillain-Barré syndrome, 36 girls and 32 boys at the age of 12 months up to 18 years were treated in the Department of Developmental Neurology in Poznań in 1989-1999. The incidence of neurological symptoms was preceded in 50 patients (74%) by the infection of the alimentary or upper respiratory tracts. However, only 39 (57%) presented virus etiology and moreover, 7 children out of this group showed significant growth of antibodies to poliovirus. Six-degree range was applied to evaluate the level of motor insufficiency which involved flaccid paralysis of various degree in 48 children and adolescents. Additionally in 14 the coincidence of bulbar syndrome and i 5 patients the respiratory muscle paralysis was found. There was only 1 child with Miller-Fisher syndrome. The clinical course of the disease was divided into three phases: increase, stabilization and subsidence of symptoms. There were also presented the methods of treatment of Guillain-Barré syndrome. Immunoglobulins were applied to 9 children, plasmapheresis was administered to 8 children. Steroids were used for 37 patients and mechanical ventilation for 5 children. Motor rehabilitation was applied to all patients. The influence of the treatment method on clinical course of the disease and its final effect were evaluated. It was established that, the early administration of immunoglobulins in the form of intravenous infusions into the treatment shortens the period of paretic intensification. Plasmapheresis applied to patients with the most severe paralysis significantly reduced the period of increase and stabilisation of neurological symptoms, enabling early rehabilitation and faster mobilisation of the patients. The application of steroids did not have significant influence on the course and the final effect of treatment of Guillain-Barré syndrome.

In this study, we measured VEMP in patients with disturbed function of inner ear. We studied 4 patients with unilateral sensorineural deafness and normal excitability of vestibular organs; 6 persons with unilateral weakness of vestibular function and 10 healthy subjects. We found that in patients with unilateral weakness of vestibular function VEMPs had short latencies and diminished amplitudes whereas in patients with unilateral deafness VEMPs were unchanged. These data strongly suggested that VEMPs were generated by stimulation of vestibular and not cochlear part of inner ear-sacculus. Our data confirm that VEMPs are a highly objective technique, simple, secure and comfortable for patients to assess the vestibulo-spinal connections.

We present a case of 47-year old patient with a rare form of focal dystonia restricted to laryngeal adductors with blepharospasm. Apart from typical symptoms of blepharospasm, the patient had severe problems with articulation in the form of harsh voice, frequently interrupted speech and the sound coming out with a great effort. We applied a transcutaneous botulin toxin therapy to this patient. The toxin was given into thyroarytenoid muscle in transcutaneous injections under control of EMG. Successful clinical effect was achieved with the dose of 80 U of the botulin toxin (Dysport) and lasted 6 months. The treatment was repeated, and the patient has not presented the symptoms of the disease since then for 10 months. We confirm that the botuline toxin transcutaneous injections represent a save and effective treatment for laryngeal adductor dystonia.

In five provinces of Western Poland the incidence and prevalence of multiple sclerosis was analyzed in patients living at the place of birth and in patients who had changed their place of residence in these provinces. The results showed that the occurrence of multiple sclerosis in geographic foci is more evident if all patients living in a given area are considered, both those born there and those who had come from other localities.