Clinicoradiological changes of brain NK/T cell lymphoma manifesting pure akinesia: a case report
© Ishihara et al; licensee BioMed Central Ltd. 2011
Received: 1 August 2011
Accepted: 2 November 2011
Published: 2 November 2011
Pure akinesia (PA) is a distinct form of parkinsonism characterized by freezing phenomena. Little is known about brain tumor-associated PA. We highlight the clinicoradiological changes in a patient with PA and central nervous system (CNS) metastases of natural killer/T-cell lymphoma (NKTL).
A 68-year-old man with stage IVB extranodal NKTL developed a gait disturbance. Neurological examination of his gait revealed freezing, start hesitation, short step, forward flexion posture, festination and postural instability. Mild facial hypomimia and micrographia were observed. There was no rigidity or tremor in any of the four extremities. Brain magnetic resonance imaging (MRI) displayed T2-hyperintense lesions in the dorsal brainstem, cerebellum and periventricular white matter. Diffusion-weighted imaging (DWI) and the apparent diffusion coefficient (ADC) revealed hyperintensity in these regions. Cerebrospinal fluid cytology revealed CD56-positive cells on immunohistochemical staining. The patient's neurological deficits did not respond to L-dopa treatment and intrathecal administration of methotrexate (MTX). Two weeks later, he displayed confusion and generalized convulsions. T2-hyperintense lesions spread to the basal ganglia and the infratentorial regions. Gadolinium enhancement was observed in the cerebellum and frontal subcortex. DWI and the ADC revealed diffusion-restricted lesions in the middle cerebellar peduncles, left internal capsules and cerebral white matter. MTX pulse therapy and intrathecal administration of cytosine arabinoside and MTX were performed. Two months later, his ambulatory state was normalized. Brain MRI also revealed marked alleviation of the infratentorial and supratentorial lesions.
The clinicoradiological profile of our patient suggested that dorsal ponto-mesencephalic lesions could contribute to the pathogenesis of PA. Physicians should pay more attention to striking CNS seeding of metastatic NKTL. MTX pulse therapy had an excellent effect in improving serious symptoms and brain lesions in our patient.
Pure akinesia (PA) is a distinct form of parkinsonism that was first reported in 1972 as freezing phenomena only, characterized by frozen gait, micrographia and festinating speech . Limb rigidity and tremor were not observed. L-dopa treatment had no effects in PA patients [2, 3]. Many studies of neoplastic parkinsonism have been reported [4–14]. However, little is known about brain tumor-associated PA [4, 5]. We highlight the clinicoradiological changes in a unique patient with PA and central nervous system (CNS) metastasis of natural killer/T cell lymphoma (NKTL).
We reported a patient with CNS metastases of NKTL who had a transient course of PA and striking changes in the brain lesions.
The frequency of CNS metastases is 5-9% in patients with systemic non-Hodgkin lymphoma. Most patients have leptomeningeal or spinal epidural metastases. The rate of CNS parenchymal metastases is only 1% . NKTL is a rare type of lymphoma that usually presents as destructive lesions within the nasal cavity. Nasal NKTL frequently involves extranodal sites such as the lung, skin and CNS . CNS invasion or metastasis is common in patients with nasal NKTL . Most NKTL patients have aggressive courses and unfavorable clinical outcomes. During PA onset, our patient showed lesions in the brainstem and periventricular white matter on MRI. After he exhibited confusion and generalized convulsion, T2-hyperintense lesions were observed in the middle cerebellar peduncles and basal ganglia. DWI and the ADC revealed gadolinium-enhanced and -nonenhanced diffusion-restricted lesions in the middle cerebellar peduncles, brainstem and cerebral white matter. These neuroradiological features suggested the rapid relapse of brain NKTL. Extensive MRI lesions in the cerebral white matter have previously been described in a patient with nasal NKTL . Recent neuroradiological studies have pointed out the inverse relationship between ADC and the cell density of B cell lymphoma [19, 20]. The clinicoradiological worsening of our patient might have resulted from the fulminant progression of CNS metastases of NKTL and cerebral edema.
Previous literature of brainstem tumor-related parkinsonism
Authors [*] (years)
Gherardit et al  (1985)
B cell lymphoma
Cicarelli et al  (1999)
Yoshimura et al  (2002)
Lin et al  (2010)
B cell lymphoma
Hatano et al  (2011)
B cell lymphoma
Midbrain, hypothalamus, pineal body, thalamus, pallidum
Wächter et al  (2011)
Pons, midbrain, thalamus
Pons, midbrain, cerebellum cerebral white matter
With respect to the pathogenesis of PA, previous pathological studies have shown that some PA patients had similar lesions to those of progressive supranuclear palsy (PSP) patients [21–23]. Neuroradiological studies have also reported matching results between PA and PSP patients [24, 25]. Only two patients with neoplastic PA have been reported [4, 5]. Suzuki et al.  described a 44-year-old man with primary CNS reticulum cell sarcoma. Pramstaller et al.  reported the case of a 75-year-old man with primary CNS B cell lymphoma. This patient did not respond to L-dopa treatment, and autopsy demonstrated the involvement of the bilateral globus pallidus . The precise pathophysiological mechanism of PA remains unclear. In general, PA might contribute to both presynaptic and postsynaptic damage in nigrostriatal dopaminergic neurons [24, 25]. The midbrain tegmentum is the most common lesion site between PA and PSP patients [21–23]. A dorsorostral midbrain lesion was reported to cause PSP-like symptoms in a patient who had normal nigrostriatal dopaminergic function after resection of a pineal gland tumor . Therefore, the neuroradiological changes of our patient supported the concept that damage of the ponto-mesencephalic tegmentum could contribute to the pathogenesis of PA.
We highlighted the clinicoradiological course in a patient with transient PA and CNS metastases of NKTL. The neuroradiological changes of the present patient suggest that physicians should pay more attention to fulminant progression of CNS metastases in NKTL patients. MTX pulse therapy dramatically improved the serious neurological deficits and brain lesions in our patient.
Informed consent was obtained from the patient and his spouse for publication of this case report and any accompanying images.
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