Hemidystonia as an Initial Manifestation of Leptomeningeal Metastasis

Article information

JMD. 2009;2(2):82-85
Publication date (electronic) : 2009 October 30
doi : https://doi.org/10.14802/jmd.09022
Department of Neurology, Chonnam National University Medical School, Gwangju, Korea
Corresponding author: Seong-Min Choi, MD, Department of Neurology, Chonnam National University Medical School, 8 Hak 1-dong, Dong-gu, Gwangju 501-746, Korea, Tel +82-62-220-6171, Fax +82-62-228-3461, E-mail movement@chonnam.ac.kr
Received 2009 September 28; Revised 2009 October 23; Accepted 2009 November 02.

Abstract

A 76-year-old woman gradually developed action dystonia of the left hand and foot. Leptomeningeal metastasis of the right fronto-parietal area associated with gastric adenocarcinoma was found on the brain magnetic resonance imaging (MRI) and positron emission tomography (PET) studies. We discuss the mechanisms involved in the development of secondary hemidystonia and review dystonia associated with cortical lesions.

Focal or hemidystonia usually appears as a consequence of lesions in the contralateral basal ganglia and thalamus.1 Reports of cases with hemidystonia emphasize the role of the basal ganglia in the genesis of secondary dystonia. Recently several cases of secondary dystonia due to cortical lesions have been reported.2 Cortical lesions resulting in dystonia were usually described as destructive,2 and in such cases the dystonia frequently develops as a delayed phenomena.3,4 Leptomeningeal metastasis (LM) is a complication of cancer. The neurological signs and symptoms of LM are associated with the involved neuraxis.5 A case of action myoclonus was reported as an early clinical sign of leptomeningeal carcinomatosis.6 However, other movement disorders such as dystonia have not yet been reported.

We report a patient presenting with hemidystonia as the initial manifestation of leptomeningeal metastasis associated with gastric adenocarcinoma.

Case Report

A 76-year-old woman was admitted to our hospital because of involuntary movement of the left distal extremities. Two months earlier, she reported a peculiar feeling and occasional jerky and twisting movements of the left foot and hand when attempting to put on shoes or catch objects. The symptoms worsened over the next two months making fine motor tasks difficult. The patient had a past history of hypertension and endometrial cancer that was treated.

On initial examination, we found dystonic movement of the left hand and foot mainly occurring with action. On neurological examination, motor and sensation functions were preserved, but there were increased deep tendon reflexes, a positive Barbinski’s reflex and ankle clonus in the left extremities. The gait was small based and dystonia of the left foot was observed on initiation of gait.

The brain MRI revealed hyperintense lesions with irregular and nodular meningeal enhancement in the right fronto-parietal lobe, suggesting leptomeningeal metastasis (Figure 1). The MR angiography, venography and spectroscopy were non-specific. The electroencephalogram for a differential diagnosis of simple partial seizure showed no epileptic activity. The cerebrospinal fluid (CSF) contained 54 white blood cells per uL (47% lymphocyte dominant); the concentrations of protein and glucose were 79 mg/dL and 65 mg/dL, respectively. All laboratory tests including tumor marker studies and CSF cytology were normal.

Figure 1.

Brain MR showed hyperintensity and focal thickening of the right superior, middle frontal gyri and postcentral gyrus on the T2-weighted image (black arrow)(A) and fluid attenuated inversion recovery (FLAIR) image (B). Enhanced brain MR showed irregular and nodular enhancement of the right precentral and central sulci and meningeal enhancement along the right interhemispheric fissure (black arrow head)(C and F). T2-weighted and FLAIR image of the basal ganglia and thalamus showed no significant abnormal findings except for multiple small vessel disease (D and E).

The search for a hidden primary malignancy included a whole body 18F-fluorodeoxyglucose positton emission tomography (18F-FDG PET). Torso-PET scanning showed hypermetabolic signals in the gastric antrum and left upper lung, and the brain-PET scan showed hypermetabolic signals in the right fronto-parietal lobe, consistent with the brain MRI findings (Figure 2). Endoscopic gastroduodenoscopy revealed a very large ulcerated infiltrating mass at the gastric antrum, and the biopsy showed an adenocarcinoma. Finally, the patient was diagnosed with advanced gastric cancer and leptomeningeal metastasis.

Figure 2.

Torso FDG-PET showed hypermetabolic activity (maximum SUV: 5.7) of the gastric antrum (white arrow)(A) and left upper lobe of the lung (maximum SUV: 5.5)(white arrow head)(B). Brain FDG-PET showed hypermetabolic activity (maximum SUV: 10.2) of the right superior and middle frontal gyri and part of the interhemispheric fissure (black arrow)(C and D). FDG-PET: fluorodeoxygclucose positton emission tomography.

The patient received chemotherapy and medication with haloperidol, but the dystonia persisted. Two months later, after changing medication with clonazepam, the dystonia was slightly improved. Follow up brain MR continued to show hyperintense lesions without interval change.

Discussion

Basal ganglia dysfunction, with an imbalance between normal modulation of the direct and indirect pathways, is generally considered the basis for cortical disinhibition and abnormal motor output in the pathophysiology of dystonia.7 Consequently, secondary dystonia is usually produced by lesions in the contralateral caudate nucleus, lentiform nucleus or thalamus, or in a combination of these structures.1 These lesions are thought to cause the abnormal functioning of the cortico-striato-pallido-thalamo-cortical loop leading to enhanced excitation of the premotor cortical area.8 However, recent studies have emphasized the role of abnormal cortical function, especially in the somatosensory system, for the development of dystonia.9 Chuang and colleagues10 described that cortical lesions could be seen in up to 32% of patients with acquired hemidystonia. We identified 14 cases of symptomatic dystonia due to cortical lesions in the literature (Table 1).2,1118

Review of the literatures about secondary dystonia due to cortical lesion: 14 cases from 9 references

There is often a latency between cerebral injury and the onset of dystonia, and such delayed development of dystonia might be related to aberrant reorganization of the cortico-striato-pallido-thalamo-cortical loop after a static lesion.3,10 Though most cases of secondary dystonia due to stroke or trauma develop after a long latency from the initial brain lesion, a few cases of secondary dystonia due to neoplasm have presented as the initial manifestation of cortical lesion, similar to our case.14 Functional neuroimaging showed overactivation of the primary sensorimotor cortex and underactivation of the prefrontal motor areas with focal hand dystonia.19 It is likely that hypermetabolism in the primary sensorimotor cortex, in our case, could have played a role in the genesis of dystonia. One prior report suggested that provoking the cerebral cortex can evoke dystonia in the extremities.19

Clinical signs and symptoms of LM are usually attributable to the obstruction of normal CSF flow, local tumor infiltration into the brain or spinal cord, alterations in the metabolism of underlying nervous tissues, or a combination of these processes. The common presenting signs of LM are headache, changes in mental status, focal neurological deficits, and seizures.5 There have been few reports on movement disorders such as dystonia secondary to LM. Our patient presented with left hand and foot dystonia, as the initial clinical manifestation of leptomeningeal metastasis associated with gastric adenocarcinoma. This is the first case described in which hemidystonia was attributed to leptomeningeal metastasis.

References

1. Marsden CD, Obeso JA, Zarranz JJ, Lang AE. The anatomical basis of symptomatic hemidystonia. Brain 1985;108:463–483.
2. Burguera JA, Bataller L, Valero C. Action hand dystonia after cortical parietal infarction. Mov Disord 2001;16:1183–1185.
3. Burke RE, Fahn S, Gold AP. Delayed-onset dystonia in patients with “static” encephalopathy. J Neurol Neurosurg Psychiatry 1980;43:789–797.
4. Scott BL, Jankovic J. Delayed-onset progressive movement disorders after static brain lesions. Neurology 1996;46:68–74.
5. Grossman SA, Krabak MJ. Leptomeningeal carcinomatosis. Cancer Treat Rev 1999;25:103–119.
6. Valldeoriola F, González J, Vila N, Valls-Sole J, Sole M, Graus F, et al. Action myoclonus as an early clinical sign of carcinomatous meningitis. Mov Disord 1996;11:223–225.
7. Watts RL, Koller WC. Movement disorder: neurologic principles& practice Second Editon (Hardcorer)th ed. The McGraw-Hill Companies; 2004. p. 500.
8. Bhatia KP, Marsden CD. The behavioural and motor consequences of focal lesions of the basal ganglia in man. Brain 1994;117:859–876.
9. Tinazzi M, Rosso T, Fiaschi A. Role of the somatosensory system in primary dystonia. Mov Disord 2003;18:605–622.
10. Chuang C, Fahn S, Frucht SJ. The natural history and treatment of acquired hemidystonia: report of 33 cases and review of the literature. J Neurol Neurosurg Psychiatry 2002;72:59–67.
11. Pettigrew LC, Jankovic J. Hemidystonia: a report of 22 patients and a review of the literature. J Neurol Neurosurg Psychiatry 1985;48:650–657.
12. Friedman DI, Jankovic J, Rolak LA. Arteriovenous malformation presenting as hemidystonia. Neurology 1986;36:1590–1593.
13. Eaton JM. Hemidystonia due to subdural hematoma. Neurology 1988;38:507.
14. Krauss JK, Mohadjer M, Nobbe F, Scheremet R. Hemidystonia due to a contralateral parieto-occipital metastasis: disappearance after removal of the mass lesion. Neurology 1991;41:1519–1520.
15. Leiguarda R, Merello M, Sabe L, Starkstein S. Bromocriptine-induced dystonia in patients with aphasia and hemiparesis. Neurology 1993;43:2319–2322.
16. Lee MS, Rinne JO, Ceballos-Baumann A, Thompson PD, Marsden CD. Dystonia after head trauma. Neurology 1994;44:1374–1378.
17. Khan AA, Sussman JD. Focal dystonia after removal of a parietal meningioma. Mov Disord 2004;19:714–716.
18. Koch MW, Luijckx GJ, Leenders KL. Paroxysmal focal dystonia with sensory symptoms secondary to cortical oligoastrocytoma. J Neurol 2006;253:1227–1228.
19. Pujol J, Roset-Llobet J, Rosinés-Cubells D, Deus J, Narberhaus B, Valls-Solé J, et al. Brain cortical activation during guitar-induced hand dystonia studied by functional MRI. Neuroimage 2000;12:257–267.

Article information Continued

Figure 1.

Brain MR showed hyperintensity and focal thickening of the right superior, middle frontal gyri and postcentral gyrus on the T2-weighted image (black arrow)(A) and fluid attenuated inversion recovery (FLAIR) image (B). Enhanced brain MR showed irregular and nodular enhancement of the right precentral and central sulci and meningeal enhancement along the right interhemispheric fissure (black arrow head)(C and F). T2-weighted and FLAIR image of the basal ganglia and thalamus showed no significant abnormal findings except for multiple small vessel disease (D and E).

Figure 2.

Torso FDG-PET showed hypermetabolic activity (maximum SUV: 5.7) of the gastric antrum (white arrow)(A) and left upper lobe of the lung (maximum SUV: 5.5)(white arrow head)(B). Brain FDG-PET showed hypermetabolic activity (maximum SUV: 10.2) of the right superior and middle frontal gyri and part of the interhemispheric fissure (black arrow)(C and D). FDG-PET: fluorodeoxygclucose positton emission tomography.

Table 1.

Review of the literatures about secondary dystonia due to cortical lesion: 14 cases from 9 references

Reference Age Etiology Latency Lesion location
Pettigrew11 29 Arachnoid cyst 29 yrs Frontoparietal
Friedman12 33 AVM Unknown Temporoparietal
Eaton13 56 Traumatic SDH 10 wks Hemispheric
Krauss14 57 Tumor Unknown Parieto-occipital
Leiguarda15 (3 cases) 68 Stroke+DA 1 yrs Frontoparietal
30 Stroke+DA 3 yrs Frontoparietal
49 Stroke+DA 2 yrs Frontotemporoparietal
Lee16 (4 cases) 7 Trauma 9 yrs Hemispheric
7 Trauma 2 yrs Parietal
20 Trauma 1 yrs Occipital
44 Trauma 3 yrs Hemispheric, both
Burguera2 46 Stroke Several wks Parietal
Khan17 60 Meningioma 2 yrs Parietal
Koch18 61 Oligoastrocytoma Unknown Postcentral gyrus

AVM: arteriovenous malformation, SDH: subdural hematoma, Stroke+DA: use of dopamine agonist (bromocryptine) in stroke patients, yrs: years, wks: weaks.