Neuro-Ophthalmological Features of Primary Diffuse Leptomeningeal Gliomatosis

Abstract

We performed an in-depth study of the neuro-ophthalmologic signs and symptoms of a rare but fatal disease known as primary diffuse leptomeningeal gliomatosis (PDLG). Two new cases of PDLG are described, and 22 published cases reviewed. Papilledema and sixth nerve palsy are the most common neuro-ophthalmic findings. Other abnormalities include third and fourth nerve palsies, nystagmus, and vision loss. Involvement of the visual system may be part of the initial presentation of PDLG.

Malignant meningitis presenting as pseudotumor cerebri

“…Interventional treatment for patients with PTC from meningeal metastases should be ruled by the rate of progression of the visual loss and in those with malignant leptomeningitis by the need for intra-thecal chemotherapy [8,18]. We elected to offer ONSF to one of our patients [36,37] and ONSF and transverse sinus stenting to one [3] and it seems that they were able to retain vision for the little time that they had to live: 6 weeks in one patient and 4 months in the other.…”

“…PTC can occur in infective, fungal [6] or rickettsial [7] meningitis. In patients with primary or secondary malignant leptomeningitis ICH is usually due to, or at least attributed to, communicating hydrocephalus [8,9]. However we have found single case reports of patients with malignant leptomeningitis [10,11] or pachymeningitis (dural metastases) [12,13] who have ICH with papilledema but without hydrocephalus, but are not recognized as having the clinical syndrome of PTC.…”

“…Rare primary malignancies of the cranial or spinal leptomeninges, such as glioma [8], melanoma [14,15] or lymphoma [16][17][18] and also the more common secondary leptomeningeal malignancies [9,19,20] can all produce ICH. While it is well recognized that these patients can have hydrocephalus without papilledema, only a few have been noted to have papilledema without hydrocephalus-i.e.…”

Primary Diffuse Leptomeningeal Gliomatosis in Children

“…2 Ophthalmic manifestations of PDLG include optic disk edema or pallor, bilateral sixth nerve palsy associated with visual loss, and diplopia. [3][4][5] Neurologic symptoms of PDLG include headache, cranial nerve involvement, meningismus, and decreased mental status. 6 PDLG is a rare disease with only 26 biopsy-proven cases reported in the MEDLINE-indexed English literature, including 10 cases involving children.…”

“…2,3 Only 2 patients have been reported in the ophthalmologic literature, despite the frequent and early eye involvement in the disease. 3 The prognosis is very poor, even with treatment; in a significant percentage of cases, the correct diagnosis is established at the time of the autopsy. Differential diagnosis of PDLG includes mainly aspecific viral or tuberculous meningitis.…”