The "Child in the Barrel syndrome"--severe pharyngeal-cervical-brachial variant of Guillain-Barre Syndrome in a toddler.

One week after a flu-like prodrome, an 18-month-old boy developed acute severe, symmetrical, painless weakness and wasting of the shoulder girdle and upper limbs, drooling, dysphagia, dysarthria, atrophy and fasciculations of the tongue. Milder paresis involved the mimic muscles and the neck extensors. The legs were intact with brisk reflexes. The flail immobile upper limbs produced the appearance that the boy was restrained in a narrow barrel. Electrodiagnostic findings suggested demyelinating motor neuropathy sparing the legs. CSF (45 days after onset) was normal. Initial recovery was observed but 70 days after onset the child suffered severe relapse and died from respiratory arrest. This is another rare case of the pharyngeal-cervical-brachial variant of Guillain-Barre syndrome in infancy with an unusual relapsing course leading to a fatal outcome.

Effect of gender on orthodromic sensory nerve action potential amplitude.

OBJECTIVE: Studies in the literature have demonstrated a gender effect on sensory nerve action potential (SNAP) amplitude for the median and ulnar nerves by use of the antidromic method of recording. The objective of this study was to determine if performing orthodromic sensory nerve stimulation eliminates the gender bias by removing the finger circumference as a variable. METHODS: Fifty-five healthy subjects participated in the study. The mean age of the subjects was 37.8 +/- 5.9 yr and 35.3 +/- 5.9 yr for men and women, respectively. Orthodromic sensory nerve conduction studies were performed for the median and ulnar nerves measuring the SNAP amplitude by use of standard electrophysiologic technique. RESULTS: The mean finger circumference of the third digit was 6.5 +/- 0.58 cm for men and 5.9 +/- 0.47 cm for women, and for the fifth digit, it was 5.6 +/- 0.41 cm for men and 5.3 +/- 0.37 cm for women. The median SNAP amplitude and their percentiles of 2.5 and 97.5 for the median nerve were 30.0 microV for men and 28.0 microV for women. For the ulnar nerve, they were 16.5 microV for men and 16.0 microV for women. CONCLUSION: The study confirmed that orthodromic sensory nerve stimulation did not have any significant effect on SNAP amplitude between men and women.

Axonal and demyelinating neuropathy with reversible proximal conduction block, an unusual feature of vitamin B12 deficiency.

We report a 35-year-old patient with megaloblastic anemia who presented with features of subacute combined degeneration of the cord. Electrophysiological studies showed features of axonal neuropathy. In addition, there was evidence of prominent focal proximal conduction block in several nerves. After treatment with cyanocobalamin the neuropathy improved, and the peripheral nerve conduction block detected earlier disappeared. Reversible nerve conduction block as a feature of vitamin B12 deficiency in man, to our knowledge, has not been reported in literature, so far.

Behçet's syndrome: a report of 41 patients with emphasis on neurological manifestations.

Forty one patients with the clinical diagnosis of Behçet's syndrome from two teaching hospitals in Kuwait were studied. There were 34 male and seven female patients. Age at presentation ranged from 14 to 48 years. Neurological manifestations were present in 24 patients. Eleven patients showed evidence of increased intracranial pressure, and 10 of these had radiologically confirmed dural sinus thrombosis. Five patients presented with a meningoencephalitic or meningomyelitic picture, three with a stroke-like picture, and three with primarily brain stem signs. One patient developed trigeminal neuritis, and five patients exhibited (along with other features) variable degrees of psychological manifestations. All patients with neurological involvement were treated with steroids, and some also had courses of other immunosuppressant drugs and colchicine. The disease took a relatively benign course, except those patients with meningoencephalitic and meningomyelitic presentation, one of whom died from the disease. Those treated early had a better prognosis. The incidence of dural sinus thrombosis in this series of patients is unusually high. In most patients, the course of the disease was more favourable than reported in the literature. This may be attributed to early and aggressive treatment.

Lissencephaly associated with cerebellar hypoplasia and myoclonic epilepsy in a Bedouin kindred: a new syndrome?

Clinico-radiological assessment of three mentally retarded members of a large Bedouin kindred showed lissencephaly, spastic paraparesis, myoclonic epilepsy and cerebellar hypoplasia. It seems that the familial association of lissencephaly/myoclonic epilepsy/cerebellar hypoplasia represents a new entity.

Marinesco-Sjögren syndrome in a Bedouin family.

Marinesco-Sjögren syndrome is rarely reported in the Middle East. This is the 2nd report of Marinesco-Sjögren syndrome in an Arab family. The clinical features of 2 affected brothers are described. Electrophysiological studies of the 2 patients showed primarily myopathic changes, whereas sural nerve biopsy revealed segmental demyelination and axonal degeneration. The role of tissue biopsy and the relationship to different electrophysiological studies are discussed. Both patients were noticed to have abnormally short lateral 3 metatarsals, a feature not present in other healthy members of the family. We suggest that this feature should be considered part of the syndrome profile.

Miller Fisher syndrome: unique involvement of ophthalmic division of trigeminal nerve.

The clinical, electrophysiological, and radiological features of a patient with a typical Miller Fisher Syndrome are reported. The patient has shown a unique affection of the ophthalmic division of the trigeminal nerve. The significance of serial electrophysiological testing particularly blink reflex study is discussed.

Jitter of the stimulated motor axon.

Electrical microstimulation of motor axons in conjunction with single fiber EMG (SFEMG) is increasingly used to measure the jitter of the motor endplates. This study examines the jitter of the stimulation site on the axon when stimulus strength is at threshold. In the absence of spurious blocking, this was found to be 5 microseconds on the average. With intermittent blocking, however, a mean additional jitter of 40 microseconds was obtained. The latter is considered to result from changing propagation velocity in the muscle fiber due to irregularity of activation rate. In clinical jitter studies, inadvertent threshold stimulation can result in significant error when associated with intermittent blocking.

Brainstem auditory evoked potentials in Wilson's disease.

Twelve patients with Wilson's disease, aged 11-25 years, underwent brainstem auditory evoked potential (BAEP) study. The results were correlated to clinical, neuroradiological and laboratory data. Ten had prominent to severe neurological manifestations, suggestive of involvement of one or several CNS structures, and 2 were neurologically free. All had evidence of abnormal copper metabolism, and 8 had CT scan evidence of brain atrophy, or hypodense areas in basal ganglia, or both. The 2 patients without neurological manifestations as well as one with neurological signs had normal BAEP. One patient with neurological signs had increased N1 latency due to cochlear hearing loss, but normal interpeak intervals, while 8 of 10 patients with prominent neurological symptoms and signs had abnormal BAEPs (prolongation of NIII-NV interpeak interval). The value of NIII-NV interpeak interval correlated with the number of different neurological signs (neurological score) attributable to involvement of different CNS structures (r = 0.64 at P = 0.001). Abnormal BAEPs do not seem to be an early finding in Wilson's disease.

Brainstem auditory evoked potential (BAEP) abnormalities in brucellosis.

Twelve patients with neurobrucellosis and 17 patients with systemic brucellosis without neurological involvement underwent a brainstem auditory evoked potentials (BAEP) study. All neurobrucellosis patients (100%) showed abnormalities in their BAEP recordings, suggestive of brainstem lesions at various levels. On the other hand, only 5 (29%) of the 17 patients with systemic brucellosis had mild unilateral BAEP abnormalities, while the remaining 12 had normal responses. Comparison of pooled data between the systemic brucellosis and neurobrucellosis groups showed highly significant differences in all BAEP parameters. The recording of BAEP is thus considered a sensitive supplementary method to reveal CNS lesions in patients with neurobrucellosis.

The jitter in stimulated orbicularis oculi muscle: technique and normal values.

A technique is described of measuring the motor end plate jitter in the orbicularis oculi muscle activated by extra-muscular nerve stimulation, standardised for routine use in diagnosis and evaluation of neuromuscular transmission disorders. Among the advantages of the technique are comparatively quick and easy sampling of adequate number of single motor end-plates, convenient control of discharge rate between less than 1 and up to 20 Hz, absence of direct muscle fibre responses, and little discomfort for the patient. The jitter in the orbicularis oculi is significantly lower than that in the extensor digitorum communis muscle. There is a positive correlation between the jitter and the latency of the individual muscle fibre responses, possibly indicating that smaller diameter muscle fibres have slightly lower safety factor of neuromuscular transmission. The data collected in a group of healthy volunteers suggest the following upper normal limits for the mean of absolute consecutive difference of the latency: 30 microseconds for individual motor end plates (one out of 20 values may be higher), and 18 microseconds for a median of 20 motor end plates.

Readiness potential of cortical area 6 preceding self paced movement in Parkinson's disease.

The vertex Readiness Potential (RP) (Bereitschaftspotential) preceding self paced voluntary movements is reduced in duration and amplitude in patients with Parkinsonism. It cannot be used as an index for delayed initiation of movement. The negative potential is commonly replaced by a positive slow wave. In hemi-Parkinsonism, the abnormal RP is more apparent on moving the affected limb. The abnormal RP is related to the severity but not to the duration of Parkinsonism. Short-term fluctuations of akinesia and rigidity, spontaneous or caused by levodopa are associated with changes of RP duration and amplitude. The abnormality does not correlate with reaction or movement times. It is suggested that the Readiness Potential is a diffuse response of area 6 cortex (especially Supplementary Motor Area) evoked by pallido-thalamo-cortical afferents under dopaminergic control. It is not obligatory for pre-programming of voluntary movement. The authors postulate a dendritic potential of basket cells associated with a pallidal activated gate control of postural set.