Samuel F. Ciricillo and Mark L. Rosenblum
✓ To explore the potential usefulness of imaging studies in the diagnosis of focal central nervous system (CNS) lesions associated with acquired immunodeficiency syndrome (AIDS), the authors retrospectively examined the radiographic studies of 149 AIDS patients who presented with signs and symptoms of the three most common focal CNS lesions. Of these patients, 74 (50%) had Toxoplasma abscesses, 45 (30%) had primary CNS lymphoma, and 30 patients (20%) had progressive multifocal leukoencephalopathy (PML). Magnetic resonance (MR) imaging was more sensitive than computerized tomography (CT) in detecting lesions, especially in cases of PML. Whereas CT was unable to distinguish mass lesions caused by toxoplasmosis from those caused by lymphoma, 71% of the solitary lesions seen on MR images were lymphomas. These results indicate that empirical treatment for toxoplasmosis, the most common initial treatment for AIDS patients with neurological symptoms stemming from mass lesions, is not likely to be successful for patients with solitary lesions on MR images. Rather, early biopsy is advisable. If the presence of lymphoma is confirmed, the rapid initiation of treatment can allow prolonged high-quality survival.
James E. Boggan, Mark L. Rosenblum, and Charles B. Wilson
✓ A tumor of the trochlear nerve sheath with an unusual but diagnostic presentation is described. The rarity of reported cases may reflect failure to differentiate tumors originating from the trochlear and trigeminal nerves.
Norman P. Markowitz and Mark L. Rosenblum
Report of three cases
Adam N. Mamelak, William M. Kelly, Richard L. Davis, and Mark L. Rosenblum
✓ Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. These lesions typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. They occur in patients of all age groups; the peak incidence is in the sixth decade. Hypertrophic cranial pachymeningitis is best identified by magnetic resonance imaging. The diagnosis is established by excluding all other granulomatous and infectious diseases. A dural biopsy is essential to confirm the diagnosis. Hypertrophic cranial pachymeningitis is initially responsive to steroid therapy, but in most cases it recurs or progresses despite treatment. Surgical excision of granulomas is occasionally necessary to alleviate a mass effect. The long-term outcome remains uncertain for most patients, but progressive disease is usually fatal owing to cranial neuropathies.
Takao Hoshino, Charles B. Wilson, Mark L. Rosenblum, and Marvin Barker
✓Four patients received 3H-thymidine 4 to 7 days and vinblastine 4 to 6 hours prior to operation for recurrent malignant gliomas (three glioblastomas and one anaplastic astrocytoma). Tumor biopsies obtained at operation were fixed for routine histological studies and radioautography. The tumors' growth fractions averaged 0.28 with a range of 0.14 to 0.39. The tumor cell cycle time calculated in three patients had a mean duration of 57 hours with a standard deviation of 6 hours. The authors concluded that: 1) single short-term courses of cell-cycle specific chemotherapeutic agents alone will probably fail to achieve either significant reduction in tumor mass or dramatic clinical improvement; 2) cell-cycle phase-specific drugs should be administered to maintain effective blood levels over 2 to 3 days for maximal tumor cell kill. Tumor growth rate appears to correlate with the fraction of proliferating cells rather than the length of the tumor cell cycle. The scientific basis for combination drug and multimodality therapy is discussed.
Robert M. Levy, Dale E. Bredesen, and Mark L. Rosenblum
✓ In this review of the acquired immunodeficiency syndrome (AIDS), the authors have evaluated a total of 352 homosexual patients with AIDS or generalized lymphadenopathy managed at the University of California, San Francisco (UCSF), between 1979 and 1984. Of an initial unselected group of 318 patients, 124 (39%) were neurologically symptomatic, and one-third already had their neurological complaints at the time of presentation. An additional 210 AIDS patients with neurological symptoms have been reported in the literature. Thus, a total of 366 neurologically symptomatic patients with AIDS or lymphadenopathy are reviewed.
Central nervous system (CNS) complications, encountered in 315 patients, included the following viral syndromes: subacute encephalitis (54), atypical aseptic meningitis (21), herpes simplex encephalitis (nine), progressive multifocal leukoencephalopathy (six), viral myelitis (three), and varicella-zoster encephalitis (one). Non-viral infections were caused by Toxoplasma gondii (103), Cryptococcus neoformans (41), Candida albicans (six), Mycobacteria (six), Treponema pallidum (two), coccidioidomycosis (one), Mycobacterium tuberculosis (one), Aspergillus fumigatus (one), and Escherichia coli (one). Neoplasms included primary CNS lymphoma (15), systemic lymphoma with CNS involvement (12), and metastatic Kaposi's sarcoma (three). Cerebrovascular complications were seen in four patients with hemorrhage and five with infarction. Five patients in the UCSF series had multiple intracranial pathologies, including two cases of simultaneous Toxoplasma gondii infections and primary CNS lymphoma, two cases of coexistent Toxoplasma gondii and viral infections, and one case of combined Toxoplasma gondii and atypical mycobacterial infection.
Cranial or peripheral nerve complications, seen in 51 patients, included cranial nerve syndromes secondary to chronic inflammatory polyneuropathy (five), lymphoma (five), and Bell's palsy (five). Peripheral nerve syndromes included chronic inflammatory polyneuropathy (12), distal symmetrical neuropathy (13), herpes zoster radiculitis (six), persistent myalgias (two), myopathy (two), and polymyositis (one).
In light of the protean behavior of AIDS and the problems related to the clinical, radiological, and serological diagnosis of the unusual and varied associated nervous system diseases, patients with AIDS and neurological complaints require a rigorous and detailed evaluation. The authors' experience suggests that biopsy of all CNS space-occupying lesions should be performed for tissue diagnosis prior to the institution of other therapies.
Brian T. Andrews, Philip R. Weinstein, Mark L. Rosenblum, and Nicholas M. Barbaro
✓ Five patients had intradural arachnoid cysts of the thoracic spinal canal associated with syringomyelia or posttraumatic intramedullary spinal cord cysts. Three cases were diagnosed 6 to 18 years after spinal surgery and two 14 to 17 years after spinal cord trauma. In each case, delayed progression of symptoms led to the identification of the lesions. The diagnosis was assisted by the use of myelography and delayed computerized tomography scanning in two cases and by magnetic resonance imaging in all five. In each case, the arachnoid cyst appeared to compress the spinal cord or nerve roots; in three cases, the syrinx cavities appeared to exert a significant mass effect. In the two trauma-related cases, the intramedullary cysts were small and may have represented areas of cystic myelomalacia. In four cases, intraoperative real-time ultrasonography helped to localize the arachnoid and intramedullary cavities. All five patients were treated by fenestration of the arachnoid cyst; additional peritoneal shunting of the cyst was performed in one case and of the intramedullary cavity in three. In one patient, the two lesions appeared to have a balancing effect; after drainage of the arachnoid cyst, the syrinx cavity expanded and had to be treated separately. The neurological deficits were reduced in four patients and stabilized in one. Intradural arachnoid cysts and intramedullary cysts may occur together as a late complication of spinal surgery or spinal cord trauma, and either or both lesions may cause delayed neurological deterioration.
S. Ather Enam, Mark L. Rosenblum, and Khang-Loon Ho
✓ A neurocytoma is a central nervous system tumor composed of small cells with features of neuronal differentiation; it typically occurs in the periventricular region, close to the septum pellucidum and the foramen of Monro. In this article, the authors report the case of a neurocytoma located in the cerebellum, which to their knowledge is the first reported case of its kind. The finding of a neurocytoma at a nonclassic location refutes the theory that this tumor has its origins in subependymal progenitor cells, unless an ectopic location of progenitor cells is invoked to explain the occurrence of a neurocytoma away from the ventricles. On the basis of this case, the authors suggest that neurocytomas should be added to the differential diagnosis of mass lesions in the supratentorial intraventricular regions as well as in the posterior fossa.