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Neuromyelitis Optica Originally Described As a Distinct 1140 Letters to the Editor J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.9.1140 on 1 September 1994. Downloaded from cerebellar connections with the brainstem, tude, as we show by the presentation of two especially the superior cerebellar peduncle, cases. should be spared for a postural type of Starting in 1981, case 1, a 28 year old tremor to occur. An intention tremor results woman, had repeatedly experienced paraes- if these connections are involved.4 The pos- thesias of the extremities and episodes of tural tremor caused by cerebellar lesions is a blurred vision. In 1988, during a further slow tremor of 4 to 5 Hz and is thought to bout of visual deterioration MRI of the result from hypotonia of the affected limbs.4 brain showed swelling and contrast The postural tremor in our case was enhancement of the optic chiasm and of the unexpected considering the location of the prechiasmatic portion of the optic nerves. mass in the thalamus with compression of An exploratory frontal craniotomy six the adjacent basal ganglia. The structures months later showed atrophic optic nerves likely to be affected by the cyst include the that were encased by abnormally thick basal ganglia and their connections, denta- arachnoidea but no tumour. torubrothalamic fibres, and the thalamic One year later the patient was first seen nuclei. Lesions of these structures are not at our department for rapid onset of left leg known to produce isolated postural weakness and bilaterally ascending hypaes- tremors. The mass in our patient could be thesia. At that time visual acuity was expected to produce a rest tremor. Even an reduced to finger counting. MRI showed intention tremor is conceivable considering swelling of the upper thoracic cord with the possible compression of the superior irregular intramedullary enhancement after cerebellar peduncle by the cyst. As men- giving gadolinium-DTPA. The brain tioned, only discrete lesions of the cerebel- seemed normal, including the chiasm. lar hemisphere have been considered to Lumbar puncture and a spinal angiogram cause postural tremors and our patient had were uninformative. Laboratory tests were neither hypotonia nor involvement of the negative for involvement of any other organ, cerebellar hemispheres. systemic infection, or collagen vascular dis- The tremor in our case was contralateral ease. No specific antibodies except for an to the side of the tumour and responded to intermittently raised titre against Toxo- aspiration of the cyst contents, suggesting plasma gondii was found. that the pressure exerted by the cyst on Over the next four years the patient con- adjacent neural structures was in some way tinued to have bouts of myelitis at times responsible for the production of the involving almost the entire spinal cord, as tremor. To the best of our knowledge there shown by MRI. Cell counts in the CSF has been no previous report of unilateral ranged from 0 to 10/mm3 and consisted postural tremor caused by a thalamic or mainly of lymphocytes and activated basal ganglia lesion. monocytes. Sometimes fat containing VEDANTAM RAJSHEKHAR macrophages indicating tissue necrosis were Department ofNeurological Sciences, Christian Medical College and Hospital, also seen. The protein content of CSF was Vellore 632004, India mostly increased and ranged from 71 to 153 mg/100 ml, but there was no evidence of 1 Friede RL, Yasargil MG. Supratentorial intrathecal immunoglobulin production. intracerebral epithelial (ependymal) cysts: for review, case reports and fine structure. Repeated search oligoclonal bands was Neurol Neurosurg Psychiatry 1977;40: negative. 127-37. Various therapeutic regimens including 2 de Yebenes GJ, Gervas JJ, Iglesias J, et al. high dose steroids, immunoglobulins, Biochemical findings in a case of parkinson- ism secondary to brain tumor. Ann Neurol immune adsorption, and antibiotic treat- 1981;11:3 13-6. ments failed to stabilise the patient's clinical T 1 weighted axial (A) and sagittal (B) MRI 3 Liversedge LA. Involuntary movements. In: condition. When she was last seen in mid- of the patient showing the hypointense cystic Vinken PJ, Bruyn GW, eds. Handbook of 1992 she was bedridden because of a lesion in the right thalamus and basal ganglia. clinical neurology, Vol 1. Amsterdam: North Compression of the upper midbrain by the mass Holland Publishing Co, 1969:277-92. spastic tetraparesis with a bilateral http://jnnp.bmj.com/ is evident in the sagittal scan. 4 Lang AE. Movement disorder symptomatol- sensory level at C2 and had unchanged ogy. In: Bradley WG, Daroff RB, Fenichel severe visual impairment. MRI of the brain GM, Marsden CD, eds. Neurology in clinical practice, Vol 1. Boston: Butterworths; continued to be normal whereas the spinal Heinemann, 1991:315-36. cord at the cervical and upper thoracic At follow up three months after surgery, region was very atrophic. The patient the patient was asymptomatic and had no refused the administration of contrast tremors of the left upper or lower limbs. A material at that time. small residual cyst was seen on CT but it For five months case 2, a 22 year old was not producing any mass effect or ven- woman, had experienced continuous deteri- on September 26, 2021 by guest. Protected copyright. tricular dilatation. No further treatment was oration of visual acuity associated with offered and she was advised clinical and CT MRI of neuromyelitis optica: evidence increasing bilateral leg weakness and uri- monitoring. for a distinct entity nary incontinence before she was seen at Intracranial tumours are on occasions our department. Visual field testing showed known to produce a parkinsonian syndrome Neuromyelitis optica was originally concentric narrowing on the right and a with rest tremors, bradykinesia, and described as a distinct demyelinating disor- temporal hemianopia on the left with spar- rigidity.23 Another type of tremor that has der characterised by visual disturbances and ing of the fovea. Her right arm distal to the been described in association with intra- spinal cord signs occurring closely in time.' elbow was mildly hypaesthetic and sensa- cranial masses is "rubral tremor". Involve- Later studies on patients presenting with tion to pinprick and pain was reduced dis- ment of the red nucleus and the decussating this syndrome often reported the subse- tally from T7 bilaterally. The plantar fibres of the superior cerebellar peduncle by quent evolution of additional neurological response was upgoing on both sides. A lesions in the midbrain region has been deficits,2 found pathological evidence for a spinal tap gave a colourless CSF with 4 implicated in the production of "rubral more widespread demyelination, or dis- lymphocytes/mm' and a protein concentra- tremor".4 In both these instances, the closed other specific aetiologies such as tion of 50 mg/100 ml. There was no evi- tremor is present at rest, although rubral acute disseminated encephalomyelitis, dence of oligoclonal bands. MRI showed tremors may be aggravated by maintenance Behcet's disease, or systemic lupus erythe- right sided enlargement of the chiasm and of a posture or goal directed movement. matosus.3 From these findings it was con- of the right optic nerve with uptake of con- Postural tremors can theoretically occur cluded by many that neuromyelitis optica trast material. The remainder of the brain in isolated cerebellar hemispheric lesions was inseparable from multiple sclerosis and was normal. There was also minimal including mass lesions, but this manifesta- its existence as a specific entity was ques- swelling of the cervical medulla and patchy, tion is rarely seen in clinical practice. The tioned.34 MRI promises to alter this atti- intramedullary enhancement from Cl to Letters to the Editor 1 141 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.57.9.1140 on 1 September 1994. Downloaded from C4. Laboratory tests for specific causes of M myelitis were negative. The patient was I tf- given steroids but improvement was mini- mal. A repeat MRI two months later showed a further increase in chiasmal pathology with extension into both optic nerves (fig 1). Spinal cord involvement seemed almost unchanged (fig 2). After a waxing and wan- ing of symptoms for the next 18 months her last MRI confirmed still active optochias- matic and spinal cord disease which spared the remainder of the CNS. The MRI findings in both our patients exactly parallelled the pattern of damage described as characteristic for neuromyelitis optica in pathological studies.5 They con- sisted of swelling and congestion of the optochiasmatic region and the spinal cord due to severe demyelination which often progresses to whole tissue necrosis. Medullary inflammation extended over many segments, tended to involve both grey and white matter, and had a predilection for the cervical and upper thoracic cord. The brain itself remained free from demyelina- tion. This pattern was very different from the distribution of lesions found in multiple sclerosis. There MRI typically shows numerous round or oval signal hyper- intensities, which are scattered throughout the brain with a predilection for the periventricular region.6 Extensive spinal cord damage seen on MRI as in our patients has already been described in four other patients with sus- pected neuromyelitis optica.57 The MRI fol- low up of optic neuritis attributed to Figure 1 Case 2: Magnification of Tl weighted coronal cuts through the chiasm (left) and optic neuromyelitis optica has also been nerves (right) before (upper panel) and after (lower panel) application ofgadolinium-DTPA. There reported.8 Yet our study for the first time is pronounced asymmetric swelling of the chiasm with contrast enhancement extending into both optic presents the full diagnostic potential of MRI nerves (arrows). for this disorder-that is, to show disease activity in both optic chiasm and spinal cord of the same patient and to rule out other abnormalities in the brain. It is also impor- tant to stress that the disease of our patients remained limited strictly to the optic nerves, chiasm, and spinal cord during a relapsing- remitting course of at least seven and two years, respectively.
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