CSF sulfoconjugated catecholamines in man: their relationship with plasma catecholamines.

Simultaneous plasma and cerebrospinal free and sulfoconjugated norepinephrine, epinephrine, dopamine and homovanillic acid determinations in 38 patients with various neurological disorders have shown consistently lower concentrations of dopamine sulfate, norepinephrine sulfate, and epinephrine sulfate in the cerebrospinal fluid than in plasma. An approximately three fold increase of plasma dopamine sulfate concentrations following banana ingestion did not result in increased dopamine sulfate concentrations in cerebrospinal fluid. There were positive correlations between plasma and cerebrospinal fluid concentrations of free norepinephrine and epinephrine as well as norepinephrine sulfate, dopamine sulfate and free homovanillic acid. Cerebrospinal fluid and plasma are apparently separated by the blood-brain barrier impermeable to catecholamine sulfates from the blood. Some other data and the observed positive correlation between cerebrospinal fluid and plasma dopamine and norepinephrine sulfates suggests however that catecholamine sulfates may pass from cerebrospinal fluid to the blood and reflect events in the brain.

[Late post-traumatic syringomyelia. Discussion apropos an atypical case]. / Syringomyélie post-traumatique tardive? Discussion à propos d'un cas atypique.

Late post-traumatic syringomyelia is uncommon, the clinical signs appearing several years after severe trauma to the cervical spinal cord. It is due to progressive cavitation in the spinal cord. In the case reported here progress was atypical because there were no immediate neurological signs and the symptoms of syringomyelia developed only 3 weeks after injury. Several possibilities are open to discussion. Was the trauma responsible for the syringomyelia? Did it cause an aggravation of a pre-existing hydromyelia or were both lesions purely coincidental? A silicon tube draining the cavity in the cord to the subarachnoid space resulted in a real improvement in the neurological condition.

[Multiple intracranial aneurysms associated with primary hyperaldosteronism]. / Anévrysmes intracrâniens multiples associés à un hyperaldostéronisme primaire.

Multiple intracranial aneurysms have been reported in association with polycystic disease of the kidney, brain tumor, pituitary adenoma and coarctation of the aorta. We report the association of multiple aneurysms with primary hyperaldosteronism due to bilateral adrenal hyperplasia in an 18 year old left-handed man who presented with subarachnoid hemorrhage and arterial hypertension. We report the excellent outcome of this patient in spite of a difficult and surgical management. Ligation of all three intracranial aneurysms was performed after an extra-intracranial arterial bypass was done as a protective measure.

Hydrocephalus and headaches in Paget's disease of the skull: complete relief by ventriculo-atrial shunt.

A 76 year old patient with a long history of headaches was found to have Paget's disease and communicating hydrocephalus. There were (otherwise) no neurological or musculo-skeletal manifestations of Paget's disease, but moderate impairment of intellectual function was present. Treatment with disphosphonates did not bring any significant improvement, but three days following a ventriculo-atrial shunting procedure, the patient became headache-free for the first time in several years. In the literature, patients with hydrocephalus have been shown to respond quite unevenly to atrio-ventricular shunting, but in most instances the descriptions concerned advanced cases with well-established symptoms of dementia, ataxia and incontinence. Our case is reported to stress the importance of early diagnosis and management of hydrocephalus in Paget's disease for the prevention of widespread neurological dysfunction.

Common variable hypogammaglobulinemia in children. Clinical and immunologic observations in 30 patients.

We made clinical and immunologic observations of 30 children with common variable hypogammaglobulinemia. The mean age at diagnosis was 10.5 years, five years after clinical onset. Diagnosis was initially made based on a history of recurrent otobronchopulmonary infections, diarrhea, or both. The most common complications included short stature, bronchiectasis, and malabsorption, often associated with giardiasis or sprue. Nine patients had associated autoimmune diseases (eg, atrophic gastritis, arthritis, and hemolytic anemia). Three patients died, one of chronic respiratory insufficiency, one of chronic persisting hepatitis, and one of osteogenic sarcoma. Humoral and cellular immune functions of all patients were examined.

[Precocious puberty. Comment on the diagnostic conditions and etiological aspects]. / Pubertés précoces. Réflexion sur les conditions de diagnostic et les aspects étiologiques.

The present review is based on the retrospective study of 124 children with precocious puberty, 92 girls and 32 boys. In girls, the analysis of the clinical initial presentation has shown that premature pubarche (n = 18), premature adrenarche (n = 2) or isolated menstruations (n = 3) must be ruled out, as these symptoms can remain isolated for more than a year. However, in most cases the presence of growth acceleration and vaginal estrogenisation was of major diagnostic value. Bone maturation, although generally accelerated, can be normal in recently developed puberty. Precocious puberty may proceed by steps, with complete disappearance of physical signs in the intervals. Organic causes were found in 31% of the girls, and 44% of the boys with some characteristic features as rapid progression, and elevated LH response to LRF stimulation. Main causes were glioma of the optic chiasma (n = 11), 3rd ventricule invasive tumors, hamartoma (n = 8). The latter should be looked for by a non invasive procedure as the CT scan. In girls, precocious puberty with very high circulating estrogen levels was observed as part of a McCune-Albright syndrome. As the effect of precocious puberty on the final adult height is variable, the evaluation of therapeutic results remains uncertain. Medroxyprogesterone as well cyproterone acetate have not been fully efficient in controlling bone maturation. More recently, and still controversial, the treatment with long acting LRF analogues might provide a more satisfactory statural prognosis.

Balint's syndrome.

Balint's syndrome is usually attributed to bilateral parieto-occipital lesions. In several reported cases involvement of the frontal lobes was also documented and could be responsible for the "spasmodic fixation" often recorded in these patients. We report a case of Balint's syndrome in a patient with bilateral frontal and parieto-occipital metastases demonstrated by CT scan and confirmed by postmortem examination.

Generalized giant axonal neuropathy-a case with features of Fazio-Londe disease.

We present the 8th case so far published of generalized giant axonal neuropathy. Many features of the neurological picture of this 8 year 8 month old boy were suggestive of progressive bulbar paralysis of childhood. Muscle and sural nerve biopsies disclosed typical nodular axonal swellings measuring between 10--29 nm. Ultrastructure of the inflated portions of the axon consisted almost exclusively of 10 nm filaments. Normally appearing constituents were found in the non-dilated portions of the axons with this particularity that glycogen and mitochondria were aggregated. Previous clinical and pathological evidence indicates that this rare disorder not only affects the peripheral nerves but also the whole CNS. Our case differs from the others reported in literature by its rapidly deteriorating course and by the prominent involvement of some brain-stem motor functions; we offer the theory that a link might be implicated between this entity and Fazio-Londe disease.