Intra-cerebral relapse following prolonged remission after autologous stem cell transplantation for multiple myeloma.

Central nervous system (CNS) myeloma is a rare phenomenon, especially so after high-dose therapy (HDT) and stem cell transplantation. We describe a case of isolated CNS relapse of myeloma post autologous transplantation that followed a prolonged progression-free interval. Issues regarding the pathophysiology and management of this unusual complication are discussed.

Intraorbital rupture of a cavernous internal carotid artery aneurysm: therapeutic options.

PURPOSE: To describe the use of an endovascular therapeutic technique in the management of a giant carotid cavernous aneurysm. METHODS: We reviewed the clinical and neuroradiologic findings of a patient with an unusual case of carotid cavernous aneurysm and intraorbital rupture. The medical literature was searched for similar cases and to review the use of endovascular techniques. RESULTS: The patient was treated by balloon occlusion of the left internal carotid artery. CONCLUSIONS: Endovascular techniques can be used to treat complex giant cranioorbital cavernous aneurysms.

Communication and mental preparation issues for the epilepsy surgery patient.

This paper reviews the importance of adequate communication between the treatment team and the patient and family in epilepsy surgery. Ensuring that patient and family adequately understand the diagnosis, natural history, management alternatives and the risks and outcomes of surgery is a challenge for each epilepsy team. Guidelines as to process and content from the authors' experience and the participants in the workshop are presented.

Management-related morbidity in unselected aneurysms of the upper basilar artery.

A series of 49 consecutively treated patients with 52 aneurysms of the upper basilar artery (BA) is presented. Thirty-nine aneurysms arose at the BA bifurcation, 11 at the origin of the superior cerebellar artery (SCA), and two from the upper BA trunk just below the SCA. The patient population consisted of 36 women and 13 men, with a mean age of 50 years (range 23-74 years). Of the 35 patients presenting with subarachnoid hemorrhage, 10 were Grade I, 10 were Grade II, 11 were Grade III, and four were Grade IV according to the Hunt and Hess scale. Treatment consisted of aneurysm neck clipping in 28, proximal occlusion of the BA in three, and endovascular therapy with coils in four patients. The remaining 14 patients with unruptured aneurysms underwent direct neck clipping. Postoperatively, 38 patients developed diplopia in at least one direction of gaze but this had resolved in 31 of them at the last follow-up evaluation. There were four deaths (8.2%): two as a result of rebleeding following coil compaction at 8 days and 9 months posttreatment, respectively; one as a result of vasospasm; and one as a result of brainstem infarction after proximal occlusion of the BA in a giant bifurcation aneurysm. Of the surviving patients, 33 (67.3%) made an excellent recovery, seven (14.3%) made a good recovery, and five (10.2%) were in poor condition at the last follow-up review. Direct microsurgical clipping of most aneurysms of the BA apex region can be performed with acceptable rates of morbidity. These data from an unselected series of patients in a general hospital provide a basis for comparison with developing alternative techniques.

Reactive microglia in hippocampal sclerosis associated with human temporal lobe epilepsy.

An immunoperoxidase method was used to demonstrate expression of HLA-DR (a Class II major histocompatibility antigen) as an indicator of microglial activation in cases of hippocampal sclerosis derived from temporal lobectomy for intractable seizures. HLA-DR-immunoreactive microglia were increased approximately 11-fold in CA1 and 3-fold in CA3, compared to control autopsy hippocampus. The numbers of HLA-DR-immunoreactive perivascular cells were also significantly increased in hippocampal sclerosis cases (9-, 7- and 6-fold increases in CA1, CA3 and CA2, respectively). Since animal studies have found microglial activation to be an acute or subacute response to injury, the results presented here suggest that, contrary to the classical conception of human hippocampal sclerosis as an inert scar, neuronal injury continues to occur as a result of ongoing seizure activity.

Combined frontotemporal-orbitozygomatic approach for tumors of the sphenoid wing and orbit.

An extension of a combined frontotemporal and orbitozygomatic exposure was developed to remove 8 hyperostosing invasive sphenoid wing meningiomas (Group 1) and 11 complicated intraorbital tumors with and without intracranial extension (Group 2). Two separate bone flaps were created: a free frontotemporal-sphenoidal (pterional) bone flap and en bloc removal of the superior and lateral orbital margins with attached zygomatic arch. Cranio-orbital reconstruction was performed using the inner table of the pterional bone flap. Complete tumor removal was achieved in 14 patients and near total removal in 5. There was no mortality and in those patients who did not require orbital exenteration excellent to good cosmetic results were achieved in all but one case. This approach affords a wide exposure of the orbit and anterior and middle skull base, so that large tumors of the orbit and tumors involving the orbital apex, sphenoid wing, and infratemporal and pterygopalatine fossae can be removed.

Results of anterior corpus callosum section in 24 patients with medically intractable seizures.

Twenty-four medically refractory seizure patients, who did not qualify for excisional surgery, had anterior two-thirds corpus callosum section. Three to 11 years' postoperative follow-up suggests that this procedure can (1) lateralize a frontal lobe focus, which may lead to subsequent localized excision and (2) significantly reduce seizure frequency and severity in 75% of the patients without giving any permanent neurologic deficits. Patients with an ictal focus confined to one frontal lobe did best (8/8 improved), followed by patients with secondarily generalized seizures and multifocal bilateral foci (5/6 improved). Patients with mental retardation benefited less frequently (5/10 improved), but 4/4 from this group with ictal falls associated with Lennox-Gastaut syndrome did benefit. In this series, the improvements following the anterior partial section were lasting if present at 1 year of follow-up. Anterior corpus callosum section should be considered as a diagnostic (lateralizing) and therapeutic option in appropriately defined medically refractory patients who do not qualify for excisional surgery.

Acute subdural hematomas: atypical CT findings.

Seventy-one patients with acute subdural hematomas were examined by CT within 72 hr of a documented head injury. Lesions often did not have the classical appearance of a homogeneous, high-density extracerebral collection of blood in a crescentic configuration. Specifically, 28 patients (39%) had mixed-density subdural hematomas (MDSDH) with various degrees of low-density blood within the subdural space. In 10 of these 28 patients, the hematoma had a relatively localized mass effect with a convex inner margin, occasionally mimicking the appearance of an epidural hematoma. The MDSDH group differed from the typical homogeneous high-density subdural hematomas in that they were larger (average maximal thickness was 18.1 mm versus 8.0 mm), had more midline shift, and had a higher mortality rate (50% versus 26%). Four patients with MDSDH demonstrated an unusual pattern of ventricular compression with trapping of cerebrospinal fluid in the body of the ipsilateral ventricle and compression of the body of the contralateral ventricle. This pattern has to our knowledge not been previously described. Possible causes of the low-density regions within the hematomas include unclotted blood in an early stage of hematoma development, serum extruded during the early phase of clot retraction, or cerebrospinal fluid within the subdural space due to an arachnoid tear.

Cerebellar infarction--review of recent experiences.

Six cases of acute cerebellar infarction seen on a neurosurgery service in a general hospital during a twenty-six month period are reviewed. The clinical presentation, course, and treatment are presented and discussed. This is an important clinical syndrome which requires a high level of clinical suspicion for detection. The diagnosis rests primarily upon the clinical signs and symptoms. The C.T. Scan may provide useful confirmatory evidence and clarifies the differential diagnosis. Surgical treatment by resection of the infarcted tissue--mass lesion is urgently required for those patients who deteriorate progressively.

Carotid injury due to intraoral trauma: case report and review of the literature.

This report describes a 25-month-old child with an internal carotid artery injury that was caused by a fall upon the sharp end of a comb held in the mouth. The initial injury appeared trivial, but 24 hours later a right cerebral hemispheric infarct occurred. Delayed deterioration was caused by increased intracranial pressure and transtentorial herniation. Aggressive medical therapy was instituted for the control of intracranial pressure. The clinical and radiographic features are described in detail and the pertinent literature is reviewed. This is the first reported patient with this condition who survived once transtentorial herniation was present.

Thalamic stimulation for neuropathic pain.

Eighteen patients with neuropathic pain underwent thalamic electrode implantation. Satisfactory initial pain relief ensued in 14, and their electrode systems were internalized for long-term use. Twelve of the 14 continue to obtain either complete or partial pain relief by regular stimulation. One of the other two patients has had a complete remission of pain, apparently spontaneously, and the other had to have the electrodes removed after it retracted from his thalamus on two occasions. The electrodes have been placed in the sensory nucleus of the thalamus where stimulation evokes paresthesias in the painful part of the body. Technical problems consisting of our inability to locate the target in two patients and our failure to fix the electrode adequately in one prevented us from employing the treatment in three patients. The fourth patient had temperature dysesthesia which was not altered during 2 weeks of stimulation.

Neonatal small left colon syndrome: report of two cases.

Two cases of neonatal small left colon syndrome are described. This syndrome usually is seen as bowel obstruction in the neonate with characteristic roentgenographic features resembling those of Hirschsprung's disease. In view of the usual improvement after contrast enema, a trial of conservative management seems warranted. Rectal biopsy may be the only way to differentiate those patients who do not improve from those with Hirschsprung's disease.