Page 297 - Read Online

cord and Cauda equina [Figure 1e and f]. Opening diseases. Due to the protracted course of her illness and
pressure was normal following LP and CSF analysis the presence of multiple lesions throughout her brain
revealed lymphocytic pleocytosis (88 WBCs/uL, 94% and spinal cord, ependymoma, and neurosarcoidosis
lymphocytes), markedly elevated protein (868 mg/dL), were placed highest on the differential diagnosis.
and an ACE level of 15 U/L (normal, 0‑2.5 U/L). Cytology Several years into her disease course, her CSF profile
demonstrated lymphocytosis without malignant cells, became remarkable for the nonspecific findings of
and flow cytometry was negative for lymphoma. lymphocytosis, elevated protein and elevated ACE. [5,12,13]
The workup for infection was negative. Chest X‑ray While her CSF analysis was normal early in her disease
and CT of the abdomen and pelvis were negative for course, this has been previously reported in other cases
mass lesions or signs of systemic sarcoidosis. She of neurosarcoidosis. [6,7]
experienced only transient improvement on steroids,
so they were tapered off. Because the appropriate clinical and radiographic
findings are nonspecific, sarcoidosis diagnosis generally
Discussion at a multidisciplinary case conference requires biopsy. [1‑3] For isolated neurosarcoidosis,
yielded a broad differential diagnosis that included diagnosis is made even more challenging by the absence
inflammatory processes (neurosarcoidosis or other of thoracopulmonary involvement, a hallmark present
connective tissue diseases), primary or secondary in 90% of patients. [1,2]
CNS neoplasm (ependymoma and lymphoma), or
a chronic infectious leptomeningitis. Biopsy from Biopsy for sarcoidosis is also critical because patients
the L3 Cauda equina region was recommended as may not show substantial improvement with an
the lowest risk and highest yield target. A region of empiric trial of steroids, as was true for this patient.
a root that did not produce a motor potential when Many have advocated the use of immunosuppressants
stimulated was biopsied [Figure 1g and h]. Hematoxylin such as azathioprine, methotrexate, infliximab, or
and eosin stained sections demonstrated numerous mycophenolate mofetil alone, or in addition to steroids
noncaseating granulomas [Figure 1i]. No organisms in patients who do not respond to corticosteroid
were identified with acid‑fast or Grocott’s methenamine monotreatment. [1,2,14‑16] The risks of long‑term
silver stains. These biopsy findings support a diagnosis immunosuppressive therapy warranted a definitive
of neurosarcoidosis. diagnosis of isolated neurosarcoidosis, given its rarity.
Since neurosarcoidosis was indeed the diagnosis, the
DISCUSSION patient was initiated on 80 mg prednisone daily and
500 mg mycophenolate mofetil twice a day.
Here, we describe a case of isolated neurosarcoidosis
presenting with hydrocephalus that over three years CONCLUSION
appeared to require three separate shunt catheters,
likely due to progressive occlusion of the foramina This report highlights the importance of considering
of Monro and Sylvian aqueduct or possibly due neurosarcoidosis in the differential diagnosis in patients
to elevated CSF protein levels. Hydrocephalus with unexplained recalcitrant hydrocephalus.
is a rare symptom of neurosarcoidosis with few
case reports. [7‑10] This is, to our knowledge, the Financial support and sponsorship
first report of isolated neurosarcoidosis presenting Nil.
with hydrocephalus. Furthermore, noteworthy is
the development of a trapped lateral ventricle and Conflicts of interest
necessity of multiple shunt placements. While there There are no conflicts of interest.
has been one report of neurosarcoidosis requiring
multiple shunt placements, the patient described REFERENCES
had systemic sarcoidosis making diagnosis more 1. Hoitsma E, Faber CG, Drent M, Sharma OP. Neurosarcoidosis: a
[8]
straightforward. The protracted course of our clinical dilemma. Lancet Neurol 2004;3:397‑407.
patient’s undiagnosed illness was likely due to the 2. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med
absence of systemic disease. 2007;357:2153‑65.
3. Stern BJ, Krumholz A, Johns C, Scott P, Nissim J. Sarcoidosis and
its neurological manifestation. Arch Neurol 1985;42:909‑17.
Upon initial presentation, the diagnostic workup, 4. Nozaki K, Scott TF, Sohn M, Judson MA. Isolated neurosarcoidosis:
including CSF analysis, was unrevealing, and she was case series in 2 sarcoidosis centers. Neurologist 2012;18:373‑7.
diagnosed with idiopathic hydrocephalus at the outside 5. Sharma OP. Neurosarcoidosis: a personal perspective based on the
institution. The leptomeningeal enhancement, later study of 37 patients. Chest 1997;112:220‑8.
found on MRI, generated a broad differential diagnosis 6. Gascón‑Bayarri J, Mañá J, Martínez‑Yélamos S, Murillo O, Reñé R,
Rubio F. Neurosarcoidosis: report of 30 cases and a literature survey.
that included infectious, inflammatory, and neoplastic Eur J Intern Med 2011;22:e125‑32.

288 Neuroimmunol Neuroinflammation | Volume 2 | Issue 4 | October 15, 2015 Neuroimmunol Neuroinflammation | Volume 2 | Issue 4 | October 15, 2015 289