Parkinsonism caused by Intracranial ependymoma: A rare case report and literature review

Abstract Background Ependymomas, especially intracranial ependymomas, are rare neoplasms of the CNS. The clinical courses of patients with intracranial ependymomas can be quite variable. At present, data on parkinsonism caused by ependymomas are scarce. Case presentation A 13‐year‐old girl presented with parkinsonism symptoms of clumsiness in her left leg and hand. Her mother was diagnosed with Parkinson's disease at age 30, nine years previously. Magnetic resonance imaging showed a lesion in the temporal lobe with long‐T1 signal, mixed‐T2 signal. The patient was taken in for a right tumorectomy and was diagnosed as having an ependymoma postoperatively. The patient's symptoms fully resolved in the postoperative phase. Conclusion The case describes the mechanism of intracranial ependymoma involving parkinsonism symptoms. Our findings suggest that in some patients presenting with atypical PD symptoms the underlying cause should not be overlooked; MRI examination is necessary.


| INTRODUC TI ON
Parkinsonism is characterized by motor features such as bradykinesia, resting tremor, rigidity, postural instability and many non-motor symptoms. Several etiologies such as heredity, head injury, infection, neurotoxin, and environmental factors have been proposed as playing a role in the causes of parkinsonism. 1 The occurrence of Parkinson's syndrome is caused by dysfunction of the basal ganglia. The differentiation of idiopathic Parkinson's disease, hereditary Parkinson's disease and parkinsonism may be important for us to choose the right treatment. In this paper, we report a child with subacute extrapyramidal symptoms, whose mother was diagnosed with Parkinson's disease nine years before, resembling clinically hereditary Parkinson's disease. The patient was finally diagnosed as ependymoma by pathological examination, which was located in the right temporal lobe. To the best of our knowledge, this article is the first in the literature to document Parkinson-like symptoms in temporal ependymoma.

| C A S E PRE S ENTATI ON
A 13-year-old girl was admitted to our neurology department because of inflexibility in her left leg and left hand when she was in physical education. The patient had noticed three months previously that her left leg got easily caught up in the rope when she was skipping because of its clumsiness A similar symptom appeared in her left hand one month ago: it took a lot of effort and time for her to do up buttons.
The patient was a middle school student and had performed very well in sports three months earlier. The patient's mother had a normal pregnancy. The girl was born at 39 weeks of gestation with a birthweight of 3100 g; her Apgar score was normal.
The patient has a family history: her mother was diagnosed with Parkinson's disease at the age of 30, nine years ago, with initial symptoms of bradykinesia and rigidity in her left arm, which then progressively affected the left leg, right arm and right leg. Her mother's symptoms improved significantly after taking L-dopa 1 hour.
The patient's neurological examination showed mild bradykinesia and rigidity in her left arm and leg, her muscle strength is normal and Babinski sign is negative. Further physical examination revealed nothing abnormal: the blood pressure was 106/66 mm Hg, and her serum biochemistry, whole blood count, thyroid function were all normal. The patient had a related family history, suggesting that it might be hereditary PD. We applied genetic analysis to the proband (the patient) using targeted multiplex ligation-dependent probe amplification (MLPA)+ next-generation sequencing (NGS) to cover candidate genes known to cause familial forms of PD. We detected loplasmin (CP). The same Parkinsonism-related gene panel was applied to her mother. However, no mutation gene was found in the patient and her mother.
In addition, the patient had normal liver function, no corneal KF ring, and no ATP7B gene mutation; therefore, we did not consider this patient as hepatolenticular degeneration (Wilson disease, WD).
Moreover, the patient was not using dopamine receptor blockers, so drug-induced Parkinson's syndrome was not considered. The patient also did not have fever, personality changes, cognitive impairment, joint pain or other immune-related symptoms, autoimmune Parkinson's syndrome was not considered. She has not recently been infected or vaccinated, so infection-related Parkinson's syndrome was not considered. Moreover, she denied exposure to toxic substances, so poisoning-related Parkinson's syndrome was not considered.
Then, magnetic resonance imaging (MRI) was taken for the pa- After surgery, she received radiation therapy and there was no recurrence in the follow-up study.

| D ISCUSS I ON AND CON CLUS I ON
Ependymomas are central nervous system (CNS) tumors that usually arise from the cell lining of the ventricle and the central canal of the spinal cord or the white matter ependymal cells in the brain, accounting for 3%-5% of intracranial glial neoplasms. 2 Ependymomas can arise throughout all compartments of the central nervous system, usually involving the three major anatomic compartments (supratentorial brain, posterior fossa, and spinal cord) with prevalence for intracranial and spinal location in children and adults, respectively, which occur at two major peaks in life around 0-4 and 55-59 years of age, respectively. 3 Ependymomas mostly occur in the fourth ventricle, and may also occur in the lateral ventricle, brain parenchyma, spinal cord or cauda equine.
In adults, the majority of ependymomas are located in the spine (SP, 46%), 4 while pediatric ependymomas are almost entirely located intracranially (90%). 5 According to Cage's research, out of 182 pediatric patients, 69% had supratentorial ependymomas and 31% presented with infratentorial lesions. 6 The infratentorial extraventricular ependymomas are more often located in the cerebellar hemisphere, while the supratentorial ependymomas mainly occur in the brain parenchyma. 3 At present, data on parkinsonism caused by ependymomas are scarce. We know Kalff reported a case of Parkinson's syndrome caused by ependymoma of the cauda equine, 7 We are the first reported Parkinson's syndrome caused by ependymoma of the brain parenchyma.
The first symptom of patients with ependymomas is the intracranial hypertension for treatment. In this article, we report a case ependymoma, which usually occurs in the spine, and subependymoma that usually occur in the brain. 9 Grade II ependymoma shows pathologically perivascular pseudorosettes and characteristic true ependymal rosettes. Anaplastic ependymoma (grade III) is also called malignant ependymoma, which is characterized by hypercellularity, abundant mitotic activity, pseudoatrophic necrosis and microvascular proliferation. 10 The diagnosis of ependymoma is usually made without difficulty, but is occasionally challenging when there is a background of Parkinson's Disease (PD), because of the tumor's unusual features that include clinical manifestation and microscopically infiltrative growth and intermixed. 11 Surgery plays a major role in local tumor control. In addition to surgery, postoperative radiotherapy at a dose of 54-59.4 Gy is considered to be the standard treatment for reducing the risk of local recurrence in patients with non-disseminated ependymoma. 9,12 Thus, this case indicates that detailed medical history and imaging information were important for the accurate diagnosis of PD, especially atypical PD, which could help us choose appropriate treatment strategies for patient. Our patient's mother had similar symptoms and had been diagnosed as having Parkinson's disease for many years; then the patient presented with rapid progress of Parkinson-like symptoms, but the symptoms were atypical and genetic analysis was normal. Therefore, even if clinical symptoms, neurological examination and family history suggest hereditary Parkinson's disease, imaging examination is necessary and the underlying cause should not be overlooked.

CO N FLI C T S O F I NTE R E S T
Nothing to disclose.

AUTH O R CO NTR I B UTI O N S
Manuscript writing and interpretation of the data: YL. Acquisition and interpretation of the data: YW and LZ. Critical revision of the manuscript for intellectual content: MC and XL. All authors read and approved the final manuscript.

CO N S E NT TO PU B LI S H
Written informed consent was obtained from the patient and her mother for publication of this case report and any accompanying images.

DATA AVA I L A B I L I T Y S TAT E M E N T
All data generated during this study are available from the corresponding author upon reasonable request.