Malignant behaviour of primary intracranial Rosai Dorfman disease: A rare presentation of a benign disease.

BACKGROUND AND PURPOSE: The Rosai Dorfman disease (RDD) is a rare and usually benign lymphoproliferative disorder of unknown aetiology, typically characterized by head and neck lymphadenopathies. However, it may occasionally present with primary extra-nodal involvement including the central nervous system. We firstly described the potential malignant behaviour and fatal evolution of primary intracranial RDD. ILLUSTRATIVE CASE: A 69-year-old woman sought clinical attention for recurrent episodes of headache, fever and malaise associated with bilateral proptosis and left lower limb paresis. The brain MRI revealed bilateral retro-bulbar and fronto-parieto-occipital subdural lesions. The body CT did not show extracranial lesions. A right fronto-parietal craniotomy was performed and a subtotal resection was achieved. The histopathological diagnosis was consistent with RDD. Despite the patient was commenced on high-dose corticosteroids, she developed a worsening respiratory distress syndrome and a rapid systemic disease progression with liver and kidney failure. Given the poor general status, adjuvant medical and radiation therapies were not deemed safe and feasible and the patient died of multi-organ failure a month later. CONCLUSIONS: We documented an exceptional case of primary intracranial RDD with malignant behaviour characterized by rapid systemic disease progression and poor prognosis. Although RDD with intracranial location has usually a benign and self-limiting course with good response to adjuvant treatments, it hides a malignant potential that may lead patients to death.

Chronic bilateral electrical stimulation of the subthalamic nucleus for the treatment of advanced Parkinson's disease.

Preliminary reports in patients with Parkinson's disease (PD) showed that subthalamic nucleus (STN) stimulation was able to reverse parkinsonian state. Since 1998 we evaluated the safety and the efficacy of STN stimulation in 7 patients affected by advanced PD. All patients were included using CAPIT protocol. Motor functions and quality of life were evaluated, before and after surgery, with UPDRS and PDQ38, respectively. At the 6-month follow-up, the off medication/on stimulation UPDRS motor score improved by 50.6% and the on medication/on stimulation by 20.3%. Motor fluctuations were reduced by 57.2% and dyskinesias by 73.5%. The total L-dopa equivalent daily dose was reduced by 40.7%. PDQ38 ameliorated by 49.9%. We did not observe any perioperatory complication and only mild and tolerable side effects after stimulation.

The stereotactic volumetric information: its role in two-step resection of brainstem and thalamic giant tumor. Report of three cases and technical note.

BACKGROUND: A compact intracerebral tumoral lesion is usually considered to be completely resectable. Nevertheless, radical resection of a huge lesion located in a critical area may damage the surrounding compressed brain tissue. In cases with a good prognosis, a two-step removal appears to be a safer strategy. METHODS: In three cases, two with huge brain stem lesions and one with a thalamic lesion, a two-step volumetric stereotactic resection was planned. This strategy allowed us to evaluate the amount of tumor to be removed during the first procedure and to have, during the second operation, an exact definition of the reduced mass with regard to the scar tissue and postoperative adhesions. Furthermore, we avoided significant shifting of the cerebral structures during both procedures. RESULTS: There was a very good final recovery in the cases with brain stem lesions and a minimal deficit in the patient with the thalamic lesion. The patient with a mesencephalic lesion remained comatose for almost 2 days after the first procedure, confirming our fears about too radical a one-step resection. CONCLUSIONS: We think that by using current techniques, it is possible to remove a well circumscribed lesion regardless of its position. This is probably easier with giant lesions where a safe trajectory can be planned. In these cases, with lesions located in very critical areas but with a good prognosis, a two-step resection appears to be a good option.

Stereotactic aspiration and drainage of brain abscesses. Experience with 9 cases.

The first-line treatment for brain abscesses is still a much-debated argument. Recently, stereotactic aspiration of these lesions has gained ground as a valid alternative to the traditional medical and/or surgical therapeutical treatments. From 1991 to 1995, 9 patients affected by intracranial abscesses were surgically treated by stereotactic puncture of the lesion and drainage of pus, using the Kelly-Goerss stereotactic system. Multiple abscesses were present in one case. Specific antibiotic therapy was used in the post-operative stage, when possible. One death occurred four weeks after surgery for acute ischemia of the brain stem. Follow-up CT demonstrated gradual resolution of the abscesses in all the cases and a successful return to normal daily life in all the surviving patients. In one case, recurrence of the abscess occurred two months after resolution: a successful result was obtained by steroids and broad-spectrum antibiotic therapy alone. Possible advantages of this technique are discussed and compared with the traditional routes. The literature concerning this argument is reviewed.