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|LETTERS TO EDITOR
|Year : 2019 | Volume
| Issue : 1 | Page : 297-299
A rare extrapyramidal manifestation in a patient with primary central nervous system lymphoma
Tze Yuan Tee1, Ching Soong Khoo1, Norlinah Mohamed Ibrahim1, Syazarina Sharis Osman2
1 Neurology Unit, Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
2 Department of Radiology, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia
|Date of Web Publication||7-Mar-2019|
Dr. Tze Yuan Tee
Department of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Jalan Yaacob Latif, Bandar Tun Razak, Cheras - 56000, Wilayah Persekutuan Kuala Lumpur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Tee TY, Khoo CS, Ibrahim NM, Osman SS. A rare extrapyramidal manifestation in a patient with primary central nervous system lymphoma. Neurol India 2019;67:297-9
Primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphoma, which can involve the brain, leptomeninges, spinal cord, or eyes. PCNSL accounts for 2.2% of all primary brain tumors in the United States between years 2005 and 2009. The median age of diagnosis is 65 years with the male-to-female ratio being 1.3., The incidence of PCNSL is increasing in trend in all age groups and in both genders during the last four decades. The involvement of PCNSL in the basal ganglia is usually an incidental finding and its clinical presentation with movement disorders such as Parkinsonism More Details, dystonia, or chorea has not been widely reported.
A 61-year-old woman with diabetes mellitus, hypertension, and dyslipidemia presented to our hospital with a 3-week history of gradual unsteady gait. She was unable to walk 1 week prior to presentation. She also reported lethargy, anorexia, and weight loss. She denied fever, vomiting, back pain, or history of trauma. Upon examination, her Glasgow Coma Scale (GCS) was 15/15 with stable vital signs. The cranial nerve examination was normal. Her muscle tone was normal. She had a power of 4/5 in most of the muscles, except bilateral hip muscles, which had a power of 3/5. Reflexes were 2 + in both upper limbs and lower limbs. The plantar response was downgoing bilaterally. There were no cerebellar signs.
Magnetic resonance imaging (MRI) of her brain revealed multiple enhancing lesions at the right caudate nucleus, globus pallidus, and cerebral peduncle, which suggested the presence of central nervous system lymphoma [Figure 1]. Endoscopic brain biopsy was done and she was discharged while waiting for the results. She was readmitted 2 weeks later with left-sided body weakness, aphasia, and choking sensation for 3 days. She reported resting tremor of the right hand, which started insidiously over 1 week prior to admission. Her GCS was 12/15 (E4V2M6). She also had expressive dysphasia. Neurological examination showed that she had resting tremor of the right hand with cogwheel rigidity and bradykinesia of the right wrist. The muscle power of the left upper and lower limbs was 0/5 and right-sided body power remained 4/5. Deep tendon reflexes remained 2+ in all the limbs. Plantar reflex was upgoing on the left side. Her hematological and biochemical markers were within normal limits.
|Figure 1: Plain CT showing hyperdense lesions in the head of right caudate nucleus with surrounding hypodense edema|
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New lesions at the left globus pallidus and left cerebral peduncle were seen on the repeat brain MRI [Figure 2] and [Figure 3]. Biopsy results confirmed the diagnosis of diffuse large B-cell lymphoma (DLBCL) of a nongerminal center type. Bone marrow aspirate and trephine biopsy showed no evidence of lymphomatous infiltration. At the same time, F-18 fludeoxyglucose (FDG) whole body positron emission tomography scan showed that there was an avid signal on right-sided head of caudate nucleus indicating the presence of PCNSL with no other FDG avid disease in the rest of the body. She was then started on chemotherapy (rituximab, procarbazine, methotrexate, and vincristine). The diagnosis of tremor secondary to PCNSL affecting bilateral basal ganglia was made. The patient was started on trihexyphenidyl 2 mg BD and her resting tremor significantly reduced in intensity and frequency. She passed away due to neutropenic sepsis after the fourth cycle of chemotherapy.
|Figure 2: MRI brain (FLAIR) on coronal plane showing edema involving the left lentiform nucleus/basal ganglia|
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|Figure 3: Postgadolinium T1W MRI in axial plane showing solid enhancing lesions in the left midbrain|
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The most common type of PCNSL is DLBCL.,, According to Patel et al., who have done a 13-year retrospective study, the nongerminal center subtype was more common (which was also diagnosed in this patient) compared to the germinal center B-cell (GCB) phenotype. However, in the Western population, GCB has a higher incidence. Seventy percent of patients present with focal neurological deficits, followed by neuropsychiatric symptoms in 43%, raised intracranial pressure in 33%, and seizures in 14%., They rarely present with “B” symptoms, for example, night sweats, weight loss, and fever. According to Ferreri et al.,, 28% of the PCNSL patients have basal ganglia involvement. The most common site of involvement is the frontal lobe with 44% of patients being reported. Patients with PCNSL usually present with a single lesion. The study by Agarwal et al., reported that patients with multiple lesions were more common which was also the manifestation of this patient. Most of the PCNSL lesions do not infiltrate the basal ganglia and patients with tumors of the basal ganglia do not frequently present with extrapyramidal signs. Krauss et al., found an incidence of 0.3% of parkinsonism and resting tremor secondary to supratentorial tumors sparing the basal ganglia, in a prospective evaluation of 907 patients with supratentorial tumors. The reason postulated as to why some patients with a large tumor develop parkinsonism while some others do not, is related to the degree of loss of striatal dopamine function. Bataille et al., reported that the deeper structure involvement carries a risk of metastasis via the cerebrospinal fluid. Cerebral and cerebellar involvements have more favorable outcomes.
Yasuhara et al., conducted a review of literature and found that between 1997 and 2008, there were only 16 cases manifesting as parkinsonism related to brain tumors reported over the 11 years, of which only 2 cases were due to PCNSL. The majority of the tumors were either meningiomas ,, or astrocytomas. These tumors are often supratentorial in origin sparing the basal ganglia., Of the 16 cases, 63% of the patients were manifesting parkinsonism features initially, and most of the cases presented with more than one parkinsonism feature. In our case, her extrapyramidal manifestation occurred 1 month after the initial presentation, which corresponded to the development of new findings at the left basal ganglia visible on repeat MRI of the brain.
Kondo et al., proposed a few mechanisms related to how patients with PCNSL presented with parkinsonism: first, the basal ganglia is directly suppressed by the tumor; it is also proposed that the midbrain undergoes an upward or downward herniation. In addition, there may be distortion by the tumor, old age, loss of neurons in the substantia nigra due to tumor invasion, basal ganglia involvement by the deep-seated tumor, and damage to the connection of fibers between the basal ganglia and the supplementary motor area. The mechanism involved in our patient is believed to be due to compression of the tumor on the basal ganglia. In tumors with direct suppression of the basal ganglia and damage to the connecting fibers between the basal ganglia and the supplementary motor area, tumor removal may relieve the symptom. In case the tumor causes hernation of the midbrain and neuronal loss in the substantia nigra, the manifestations of parkinsonism might respond to the dopamine replacement. The effects of the therapy may also depend on whether the postsynaptic striatal dopaminergic receptors are preserved or not., Our patient responded well to trihexyphenidyl, which is an anti-muscarinic agent used in relieving the motor symptoms of Parkinson's disease.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]