The Neuroimaging and Clinical Spectrum of Neurofibromatosis 2

OBJECTIVE Spinal meningiomas are benign tumors with a wide spectrum of clinical and radiological features at presentation. The authors analyzed multiple clinicoradiological factors to predict recurrence and functional outcome in a cohort with a mean follow-up of more than 4 years. The authors also discuss the results of clinical studies regarding spinal meningiomas in the last 15 years. METHODS The authors retrospectively reviewed the clinical and radiological details of patients who underwent surgery for spinal tumors between 2001 and 2015 that were histopathologically confirmed as meningiomas. Demographic parameters, such as age, sex, race, and association with neurofibromatosis Type 2, were considered. Radiological parameters, such as tumor size, signal changes of spinal cord, spinal level, number of levels, location of tumor attachment, shape of tumor, and presence of dural tail/calcification, were noted. These factors were analyzed to predict recurrence and functional outcome. Furthermore, a pooled analysis was performed from 13 reports of spinal meningiomas in the last 15 years. RESULTS A total of 38 patients were included in this study. Male sex and tumors with radiological evidence of a dural tail were associated with an increased risk of recurrence at a mean follow-up of 51.2 months. Ventral or ventrolateral location, large tumors, T2 cord signal changes, and poor preoperative functional status were associated with poor functional outcome at 1-year follow-up. CONCLUSIONS Spine surgeons must be aware of the natural history and risk factors of spinal meningiomas to establish a prognosis for their patients.

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“…24 The tumor is more prevalent in the elderly population, with a peak incidence between the 6th and 8th decades.…”

Section: Discussion Demographic Featuresmentioning

confidence: 99%

Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome

Maiti

Bir

Patra

et al. 2016

FOC

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“…Its prevalence is estimated approximately at 1:60,000 [13] or less (1:87,410) [14]. The gold standard for diagnosis is the development of bilateral or unilateral vestibular schwannomas accompanied by hearing loss, tinnitus, and/or imbalance.…”

Section: Discussionmentioning

confidence: 99%

Localized Tongue Amyloidosis in a Patient with Neurofibromatosis Type II

Andreadis¹,

Poulopoulos²,

Papadopoulos³

et al. 2011

Head and Neck Pathol

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Background Localized Amyloidosis (AL) may rarely involve oral mucosa. This is the first known reported case describing the development of tongue AL in a 30-year-old patient with Neurofibromatosis (NF) type-2. Case A female patient presented with a painless, well-circumscribed nodule of the tongue. Her medical history included NF type-2 with chromosome-22 abnormal karyotype (mosaicism), multiple intracranial and spinal meningiomas/ schwannomas and unilateral blindness/deafness. The biopsy of the excised lesion of the tongue revealed subepithelial accumulation of an amorphous, nodular, fibrillar material positive for Congo red. Blood examination showed increased Thyroxine-T4 due to thyroid multinodular colloid goiter, but excluded any other hematological/immunological disorder or organ dysfunction. No recurrence was observed after a sixmonth follow-up. Conclusion This case highlights the possibility of oral manifestations as the only sign of AL and reveals the unexpected co-existence of AL and NF 2, for the first time.

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“…These include radiating pain accompanied by paresthesia, muscle weakness, atrophy, gait unsteadiness, and inturning of the foot. According to the report by Evans et al, [4] spinal tumors were discovered based on a clinical symptom (paresthesia) in only 1 (3.3%) of 30 children with NF2 aged < 10 years and Mautner et al [8] reported that spinal neurological symptoms were recognized only in 2 (4%) of 48 patients while spinal tumors were found in 43 (90%) among them. Thus, it is usually difficult to discover spinal tumors based on clinical manifestations and spinal tumors were discovered by screening MRI in most patients with NF2.…”

Section: Discussionmentioning

confidence: 99%

Bilateral lower limb paralysis as initial symptom of neurofibromatosis type 2: A case report

Yamagishi

Takahashi

Nonaka

et al. 2017

IJDI

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Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder characterized by multiple tumors including schwannomas, meningiomas and ependymomas of the central nervous system. Although paresthesias, muscle weakness, muscle atrophy, and gait unsteadiness have been reported as clinical symptoms of spinal tumors, there are no reports of patients with NF2 who developed paralysis. A 7-year-old female consulted with our hospital because of sudden bilateral lower limb paralysis. She had not recognized the abnormalities, including paralysis, paraplegia and muscle weakness of bilateral lower limb before administration. Because of the aggravated bilateral patellar tendon reflexes, Achilles tendon reflexes, and the positive bilateral Babinski reflexes, a magnetic resonance imaging (MRI) of the spinal cord was performed. MRI revealed multiple spinal tumors in the cervical, thoracic, lumbar, and sacral regions. In particular, a 12 mm × 33 mm spinal tumor was present in the thoracic region and was pressing against the spinal cord. Surgery was urgently performed to remove the spinal tumor in the thoracic region. Histopathological examination revealed that the tumor was a schwannoma. Gadolinium-enhanced MRI of the head was performed to explore the intra-cranial lesions: bilateral vestibular schwannomas and tumors in the left oculomotor nerve and right glossopharyngeal nerve were also discovered. Auditory brainstem response was performed to evaluate the bilateral VSs, and the results were normal. Nine months after the surgery, MRI of the head and spinal cord showed that her tumors had not increased in size. This represents the first reported case of NF2 complicated bilateral lower limb paralysis as initial neurological symptom.

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The Neuroimaging and Clinical Spectrum of Neurofibromatosis 2

Cited by 271 publications

References 25 publications

Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome

Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome

Localized Tongue Amyloidosis in a Patient with Neurofibromatosis Type II

Bilateral lower limb paralysis as initial symptom of neurofibromatosis type 2: A case report

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