Obstructive hydrocephalus and progressive psychosis: rare presentations of neurosarcoidosis.

BACKGROUND: Neurosarcoidosis presents with meningitis, cranial nerve involvement, and parenchymal masses. Usually, abnormal MR enhancement and/or structural lesion(s) are present. Communicating hydrocephalus arises from meningeal arachnoid granulation involvement. Reported cases of obstructive hydrocephalus have all involved obstructing ventricular lesions. CASE DESCRIPTION: A 40-year-old African American man presented with papilledema, diplopia, and headache. Magnetic resonance imaging revealed "aqueductal stenosis" without abnormal enhancement or obstructive lesion. Although symptoms resolved with shunting, he soon developed progressive psychotic symptoms. Serial MR scans remained free of abnormal enhancement or structural mass lesion(s) but revealed long repetition time and proton density signal changes within the medial temporal lobe structures and deep white matter that remained stable over 8 months despite clinical progression. Serial CSF studies were nondiagnostic. Open temporal lobe brain and meningeal biopsy revealed noncaseating granulomas within the parenchyma and meninges consistent with sarcoid. Total-body computed tomography scans ruled out systemic sarcoid. The patient steadily improved on steroid therapy. CONCLUSION: Neurosarcoid psychiatric symptoms are usually associated with diffuse meningeal enhancement from meningitis. Our case reveals that absence of abnormal enhancement or structural mass lesion on MR and normal CSF ACE levels do not rule out neurosarcoid. Based on a patent cerebral aqueduct and the T2 MR and pathology findings, we postulate that altered brain tissue compliance with impairment of normal pulsatile augmentation of aqueduct CSF flow was the likely cause of obstructive hydrocephalus. This represents a very rare psychiatric presentation and reports a new potential mechanism for the development of hydrocephalus with neurosarcoid.

Slope of the intracranial pressure waveform after traumatic brain injury.

BACKGROUND: The measurement and treatment of ICP within the management of TBI generally focuses on keeping the mean ICP to less than 20 mm Hg. More sophisticated analysis of the intracranial pressure waveform has yielded important relationships, but those methods have not gained widespread use. Prior analysis of the slope of the ICP waveform during inspiration and expiration in patients with hydrocephalus has provided valuable information that has never been applied to patients with TBI. This study used digital methods to examine ICP and the slope of the ICP waveform in relation to the respiratory cycle in subjects with TBI. METHODS: Intracranial pressure was monitored in 6 randomly selected patients admitted with acute TBI. In the first 3 subjects, a single 5-minute recording was analyzed. In 3 subsequent subjects, 4 nonsequential 5-minute epochs were analyzed during periods of varying ICP. The systolic slope of the ICP waveform was compared during inspiration and expiration, and then evaluated in relation to simultaneous mean ICP. RESULTS: The slope of the systolic ICP waveform was significantly greater during inspiration than during expiration (P < .0001 for 5 subjects and P < .03 for 1 subject). Within each subject, the ICP slope was positively correlated with simultaneous ICP (P < .0001 in all 6 cases). CONCLUSION: Greater systolic ICP waveform slope during inspiration has not been described previously after TBI and is consistent with prior observations in subjects with hydrocephalus. The strong correlation between ICP slope and simultaneous mean ICP suggests that increasing ICP slope might indicate loss of intracranial compliance after TBI.

Extramedullary hematopoeisis within a convexity meningioma.

BACKGROUND: Hematopoiesis outside the bone marrow is known to occur in patients with severe anemia, leukemia, polycythemia, or myelofibrosis, and in patients affected by chronic poisoning by marrow-toxic substances. CASE DESCRIPTION: A 66-year-old right-handed man complained of 4 days of terrible right-sided, sharp, and shooting headache for which he saw his primary care provider. Routine laboratory examination showed a WBC count of 30800/microL. Neuroimaging showed a large, right frontotemporal, extra-axial, heterogeneously enhancing, dural based mass with associated recent intramural hemorrhage with evidence of midline shift and uncal herniation. The mass was resected using a right-sided extended craniotomy with anterior fossa and middle fossa approach. A hematoxylin-eosin-stained biopsy specimen showed whorls of tumor cells, diagnostic of a meningioma. Interspersed within the tumor bulk were nucleated RBCs, representing areas of extramedullary erythropoiesis within a meningioma. Flow cytometric evaluation confirmed the clinical suspicion of an underlying chronic lymphocytic leukemia. CONCLUSION: Occurrence of extramedullary hematopoiesis within a meningioma is extremely rare. Various theories may explain the occurrence of extramedullary hematopoiesis occurring within a meningioma in our patient, such as hematopoietic differentiation of multipotent mesenchymal tumor cells; direct extension of hematopoietic activity from the neighboring marrow cavity; displacement from bone marrow of stem cells that settle and develop in tissues where capillaries and blood vessels proliferate, such as a meningioma; or congenital heterotopia of totipotent connective tissue cells, which, under certain circumstances, may transform into hematopoietic tissue.

Early propofol infusion syndrome following cerebral angiographic embolization for giant aneurysm repair. Case report.

The authors report a case of short-term high-dose propofol-related metabolic acidosis in a 3-year-old girl. The patient initially presented at another institution with left fourth cranial nerve palsy, and examination revealed a large, wide-necked 19 x 22 x 17-mm aneurysm in the left internal carotid artery. She had undergone three previous unsuccessful attempts at endovascular coil embolization, which were complicated by repeated coil protrusions into the parent vessel. During angiography and a balloon occlusion test (BOT) 80 mg propofol was given for 3 hours followed by 200 microg/kg/min for another 4 hours. The 20-minute BOT was well tolerated, and the aneurysm was occluded with stent-assisted coil embolization. Following the procedure the patient developed severe acidosis, hypotension, tachycardia, and signs of cardiac failure. On postoperative Day 3 her metabolic acidosis resolved, and she was weaned off sedatives. She continued to improve and was discharged from the hospital on postoperative Day 7. The metabolic acidosis and hypotension were thought to be due to propofol-related infusion syndrome.

Intra-arterial verapamil-induced seizures: case report and review of the literature.

BACKGROUND: Intra-arterial verapamil infusion with or without balloon angioplasty is a common therapy for patients with hypertensive, hypervolemic, and nimodipine-refractory vasospasm following aSAH. Seizures occurring from IA infusion of verapamil are rare. CASE DESCRIPTION: A 24-year-old Korean-American woman presented with aSAH from the rupture of a 5-mm ICA bifurcation aneurysm. The aneurysm was secured with clip ligation through a craniotomy, and the patient was treated with HHH therapy in the neurosurgical ICU. Routine postoperative cerebral angiography was performed to confirm occlusion of the treated aneurysm and assess for vasospasm. In the first angiogram, vasospasm was detected in the supraclinoid portion of the ICA. Intra-arterial verapamil was started; during this treatment, the patient developed right-sided focal motor seizures. The infusion was terminated and the seizures were halted with midazolam. The patient's course was unremarkable until postoperative day 7, when she developed expressive aphasia, for which she was taken for emergent cerebral angiography under anesthesia. Marked focal spasm was identified in the distal supraclinoid ICA and the left A1. The patient was treated with 25 mg of superselective verapamil infusion. Upon emerging from anesthesia, her aphasia had resolved; however, 90 minutes after angiography, she experienced generalized seizures while she was in the ICU. CONCLUSIONS: Seizures are a rare complication during cerebral angiographic procedures. Intra-arterial verapamil-induced seizures are infrequently reported. Cognizance for the potential of seizures to occur is advised during verapamil infusion for the treatment of refractory vasospasm in certain individuals.

Central causes of foot drop: rare and underappreciated differential diagnoses.

BACKGROUND/OBJECTIVE: Peripheral causes of foot drop are well recognized. However, causes stemming from the central nervous system represent rare, important, and underappreciated differential etiologies. METHODS: Two cases of foot drop stemming from central causes are described. PATIENTS: The first patient, a 46-year-old man with a remote history of lumbar spine fracture and L4-L5 instrumentation/fusion, presented with progressive weakness and numbness of the left foot, followed within 3 months by similar symptoms in the right foot. Lumbar spine imaging failed to reveal compressive nerve root pathology. Electromyography, nerve conduction studies, and muscle and nerve biopsy suggested a preganglionic lesion and ruled out a peripheral cause. Upper spine magnetic resonance imaging (MRI) revealed significant spinal stenosis at C4-C7 and T11-T12. Patient 2 was a 66-year-old man with a known left parasagittal convex meningioma diagnosed 2 years prior presented with a progressive right foot drop over 2 months. Spine imaging was normal, and serial brain MRI confirmed a slowly enlarging parasagittal meningioma. RESULTS: Following decompressive laminectomies at C4-C7 and T11-T12, patient 1's gait improved, with marked resolution of his right foot drop and significant improvement on the left. Patient 2 underwent craniotomy for microsurgical tumor resection. At the 2-week follow-up examination, he was taking daily walks. CONCLUSIONS: Central causes, although rare, need to be considered in the differential diagnosis of foot drop. Central causative lesions usually occur at locations where pyramidal tract connections are condensed and specific and the function is somatotopically organized. These cases confirm that good results can be achieved when correctable central causes of foot drop are recognized.

Delayed rebleeding of a spontaneously thrombosed aneurysm after subarachnoid hemorrhage.

BACKGROUND: This report provides a rare documentation of spontaneous thrombosis of a ruptured aneurysm followed by delayed recanalization and subsequent rerupture. CASE DESCRIPTION: A 47-year-old female presented with spontaneous subarachnoid hemorrhage (SAH). Four aneurysms were identified on CT angiogram including a basilar apex aneurysm, considered source of bleeding. Cerebral angiogram on postbleed day (PBD) #1 showed spontaneous thrombosis of basilar apex aneurysm. The patient was discharged to a nursing home on PBD #18 after two subsequent studies showed no recanalization of the basilar aneurysm. The patient returned on PBD #26 with a second episode of spontaneous SAH. The previously thrombosed basilar aneurysm had recanalized and reruptured, which was now treated with coil embolization. CONCLUSION: We are not aware of a previous report of saccular cerebral aneurysm documenting spontaneous thrombosis after SAH and recanalization with second hemorrhage. This occurrence presents a dilemma regarding the timing and frequency of subsequent cerebrovascular imaging and treatment.

Hydroxyapatite cement resistant to fragmentation following full cerebrospinal fluid bathing.

Prolonged cerebrospinal fluid bathing of cranioplasty cement frequently results in breakdown of the cement implants. A 5-year-old boy with a history of severe head trauma at 2 weeks of age presented with marked protrusion of the entire superior temporal bone and inferior parietal bone. The defect was elevated by more than 1 cm and was associated with a 4.5 x 3-cm skull defect located above and behind the right ear. There also was pulsatile tissue at the depths of the defect. A computed tomographic scan taken of the head revealed an expanding skull fracture from a dural defect with underlying brain herniation. The cranial lesion was repaired with OsteoVation hydroxyapatite cement. Within 8 weeks, the fluid encased the cranioplasty site. This resolved following implantation of a shunting device. At 2 and 12 months after the repair, the implant was still palpably solid without breakdown and did not fragment despite the prolonged bathing in cerebrospinal fluid.

Recognizing schwannomatosis and distinguishing it from neurofibromatosis type 1 or 2.

BACKGROUND DATA: Schwannomatosis has become a newly recognized classification of neurofibromatosis. Although the genetic loci are on chromosome 22, it lacks the classic bilateral vestibular schwannomas as seen in NF-2. We present the surgical treatment of 4 patients with schwannomatosis, including a brother and sister. METHOD: Case 1 presented with multiple progressively enlarging peripheral nerve sheath tumors. Case 4 presented with a trigeminal schwannoma and a vagal nerve schwannoma. Three of 4 patients had spinal intradural, extramedullary nerve sheath tumors. Surgery in all was multistaged and consisted of spinal laminectomies, site-specific explorations, and microsurgical tumor dissection and resection, with intraoperative neurophysiologic monitoring (including somatosensory-evoked and motor-evoked potentials, upper extremity electromyography and intraoperative nerve action potential monitoring, as appropriate). RESULTS: Intraoperatively the schwannomas had cystic and solid features and in all surgical cases the tumors arose from discrete fascicles of sensory nerve roots or sensory peripheral nerve branches. None of the patients experienced neurologic worsening as a result of their resections. Pathologic analysis of specimens from all cases demonstrated schwannoma. CONCLUSIONS: Not all patients with multiple schwannomas of cranial nerve, spinal nerve root, or peripheral nerve origin have NF-1 or NF-2. In schwannomatosis, these lesions are present in the absence of cutaneous stigmata, neurofibromas, vestibular schwannomas, or parenchymal brain tumors. Schwannomas in schwannomatosis can be large, cystic, and multiple. However, the predominant nerve involvement seems to be sensory and discrete fascicular in origin, facilitating microsurgical resection with minimal deficit.