Related Developmental and Epileptic Encephalopathy: Phenotypic and Genotypic Spectrum.

Background And Objectives: Purine-rich element-binding protein A () gene encodes Pur-α, a conserved protein essential for normal postnatal brain development. Recently, a syndrome characterized by intellectual disability, hypotonia, epilepsy, and dysmorphic features was suggested. The aim of this study was to define and expand the phenotypic spectrum of syndrome by collecting data, including EEG, from a large cohort of affected patients.

[Tension measurement of pulse pressure of herniation in children].

Nine patients were operated on in Department of Neurosurgery in Poznań throughout 1990-1993 because of the insufficient carotid blood circulation. In patients measurements of the pulsation pressure of the carotid artery were carried out with a tensometric sensor of pressure during surgery. Neurological symptoms, EEG, evoked potentials and other examinations were conducted before an after operation and correlated with results of pulsation pressure.

[Clinical assessment of the severity of craniocerebral injury].

Assessment of overall patient responsiveness (coma grading) is essential in the characterization of craniocerebral injuries and grading of their severity. The combination of psychological and anatomical scores and additional examinations has yielded patient characterization which more accurately relates to outcome than when those scores are used alone. The aim of this study was to survey the methods of craniocerebral injury evaluation in neurosurgical practice.

[Giant cell tumors of vertebral column].

Between 1962 and 1993 were treated at the Department of Neurosurgery, Medical Academy in Poznań 11 patients (10 women and 1 man) with giant-cell tumours of the spine. Their mean age was (26.27 +/- 17.

[Factors contributing to recurrence of intracranial meningiomas and indications to postoperative radiotherapy].

The incidence of various histological subtypes of intracranial meningiomas and recurrences was examined in 565 patients with surgically treated meningiomas in the Department of Neurosurgery in Poznań, between 1960-1992. Approximately 60% of the tumours were located on the convexity (39.5%) and parasagitally (23%).

[Central nervous system trauma in children. Clinical analysis of 782 cases].

A series of consecutive 782 children with severe head trauma and spinal cord injury who were managed surgically or conservatively in the Department of Neurosurgery, Medical Academy in Poznań during 31 years was analysed. The clinical history, severity of injury (scales AIS, ISS), outcome of head and spinal cord injuries are reviewed. Factors important in predicting a poor outcome included the presence of brainstem contusion, intracranial haematomas and complete and severe partial spinal cord lesions.

[Surgical management of pituitary adenomas by transsphenoidal approach].

In the years 1976-1993 we have performed 90 transsphenoidal approaches for pituitary adenomas. This paper presents a detail report concerning 23 cases, operated in the last 2 years, with CT and NMR documentation. A preoperative visual deficit was very common in this group of patients.

[Diagnostic significance of percutaneous needle biopsy in metastatic neoplasms of the spine].

A retrospective review of percutaneous needle biopsy due to the vertebral metastatic neoplasms performed in 92 patients treated from 1975 to 1992 at Department of Neurosurgery, K. Marcinkowski University of Medical Sciences. Thoracic biopsies constituted 45 per cent, lumbar-32 per cent, cervical-18 per cent and sacral ones 5 per cent of all procedures.

[Aneurysmal bone cyst of the skull and vertebral column treated operatively].

Aneurysmal bone cysts considered as a benign lesion are extremely rare in the skull or in the vertebral column. In the last 29-years were admitted to Department of Neurosurgery in Poznań 11 patients with aneurysmal bone cysts involving the skull in 4 and the spine in 7 cases. All but one patients were female, aged between 4 and 37 years.

[The management of subarachnoid hemorrhage in pregnancy].

Subarachnoid haemorrhage (SAH) from an intracranial aneurysm or arteriovenous malformation is a grave complication of pregnancy. Authors critically analyzed 12 cases of verified SAH during pregnancy from an 11 identified and 1 unidentified cerebral vascular malformation. Aneurysms were responsible for SAH in 7, and arteriovenous malformations in 4 patients.

[Conservative operative procedure in surgery of the spine].

An operative treatment within the spine due to the traumatic, neoplastic, vascular or other indications frequently result in complications as disturbance of the shape, flexibility, strength, mobility of the spine and injury of neural structures of a various degree. These complications may be prevented by minimizing surgical injury to the bone, ligaments, nerves and vessels and by adequate intra- and postoperative stabilization of the spine. The management will be effective only if the level, range and type of injury as well as spine tolerance against mechanical loads is diagnosed accurately.

[Spontaneous subarachnoid hemorrhage in pregnancy].

Subarachnoid haemorrhage from an intracranial aneurysm or arteriovenous malformation is a grave complication of pregnancy. The authors analyzed 11 cases of verified subarachnoid haemorrhage during pregnancy from 10 identified and 1 unidentified cerebral vascular malformation. The aneurysms were responsible for subarachnoid haemorrhage in 6, and arteriovenous malformation in 4 patients.

Determination of the glial fibrillary acidic protein in human cerebrospinal fluid and in cyst fluid of brain tumors.

The glial fibrillary acidic protein (GFAP) have been quantitatively determined in over 200 samples of liquid content of brain tumours and in cerebrospinal fluid (CSF) of cases with various tumours of the cerebral nervous system. For establishing the GFAP value, the rocket radioimmunoelectrophoresis was used. The studies were performed in three series of patients.

[Congenital carotid-sinus cervical-sagittal fistula].

The reported case presented an unusual form of congenital carotid-sagittal communication. Clinical findings included: except vascular murmur over the skull a characteristic syndrome consisting of of epleptic seizures, hemiparesis, aphasia and dementia. The communication was excised radically in its central and peripheral parts.

[Contrast ventriculography using dimer-X].

The authors report the results of 100 Dimer-X ventriculographies mainly in cases of midline tumours. The value of tomography for the diagnosis of tumours is stressed. Besides that, the contrast was used for visulization of intracerebral cavities, emptied abscesses, cavities in astrocytomas and intracerebral haematoma.

[Aneurysm situated within the optic nerve].

A case of carotid-ophthalmic aneurysm of unusual location in the optic nerve is reported. The development of the disease was sudden: pain in the eye was followed by unilaternal blindness and manifestations of subarachnoid haemorrhage. The optic nerve was dilated balloon-like, cyanotic and vigorously pulsating.

[Angiomas of blood vessels supplying the basal ganglia and the diencephalon].

The author discusses the problem of surgical treatment of intracranial angiomas situated in basal ganglia and diencephalon. Vascular malformations in this area were found in 7.5% of cases in a group of 80 patients with intracranial angiomas.