See discussions, stats, and author profiles for this publication at: https://www.researchgate.net/publication/21261126 Hemiageusia: An unusual presentation of multiple sclerosis[7] Article in Journal of Neurology Neurosurgery & Psychiatry · August 1991 DOI: 10.1136/jnnp.54.7.657 · Source: PubMed CITATIONS READS 9 23 3 authors , including: Alvaro Pascual-Leone Irfan Altafullah Harvard Medical School, Boston, MA, United States North Memorial Health Care 1,119 PUBLICATIONS 82,613 CITATIONS 29 PUBLICATIONS 1,226 CITATIONS SEE PROFILE SEE PROFILE Some of the authors of this publication are also working on these related projects: Neuroimaging of Aging in Distributed Brain Networks View project TMS a tool for the study of oscilatory activity generators View project All content following this page was uploaded by Alvaro Pascual-Leone on 01 April 2014. The user has requested enhancement of the downloaded file.
Downloaded from jnnp.bmj.com on July 15, 2011 - Published by group.bmj.com Letters to the Editor 657 Paroxysmal kinesigenic choreoathetosis 4 Wakutani K, Nakamura H, Fukuda M, Inagaki findings were a decreased right corneal reflex T, Makihara S. An autopsy case of Vogt- of multiple and hypaesthesia to pain and temperature in as presenting symptom Spielmeyer type of cerebral lipidosis. Clinical the distribution of the right mandibular sclerosis Neurology ( Tokyo) 1967;7:81-7. nerve. Cranial MRI demonstrated multiple, 5 Berkovic SF, Carpenter S, Andermann F, Andermann E, Wolfe LS. Kufs' disease: a Paroxysmal kinesigenic choreoathetosis bilateral, periventricular areas of increased critical reappraisal. Brain 1988;1 11:27-62. (PKC) is characterised by attacks of uni- or T2 signal. A similar lesion was found in the 6 Libert J, Martin JJ, Ceuterick C. Protracted and bilateral choreoathetosis precipitated by sud- right medulla on the floor of the fourth atypical forms of ceroid-lipofuscinosis. In: In the CSF myelin basic den or fast movements. The acquired form of Armstrong D, Koppang N, Rider JA, eds. ventricle (fig). Ceroid-lipofuscinosis (Batten's disease). protein was 16 2 ng/mL and five oligoclonal symptomatic PKC may be a manifestation of Amsterdam: Elsevier, 1982:42-59. underlying structural or metabolic disease.' bands without serum correlate were identi- 7 Pallis CA, Duckett S, Pearse AGE. Diffuse PKC has previously been reported in eight lipofuscinosis of the central nervous system. fied. The patient was treated with a 10 day Neurology (Minneapolis) 1967,17:381-94. course of gm/day intravenous methyl- patients as the first symptom of multiple 1 8 Vercruyssen A, Martin JJ, Ceuterick C, Jakobs sclerosis.2 We describe a patient with PKC as prednisone followed by a two week oral K, Swerts L. Adult ceroid-lipofuscinosis: prednisone taper, without improvement. the presenting symptom of multiple sclerosis, diagnostic value of biopsies and of neuro- physiological investigations. Neurol Thirteen months after presentation he in whom the lesions were localised by MRI. J Neurosurg Psychiatry 1982;45:1056-9. developed left hemiparesis. MRI A 35 year old female lawyer noticed acute 9 Dom R, Brucher JM, Ceuterick C, Carton H, demonstrated an increase in the number of diplopia and had minor attacks of sudden Martin JJ. Adult ceroid-lipofuscinosis (Kufs' periventricular lesions of increased T2 signal halts with her right foot when she started disease) in two brothers. Retinal and visceral storage in one; diagnostic muscle biopsy in the and a new right sided lesion at the expected walking as if she had "stepped into glue". She other. Acta Neuropathologica (Berlin) 1979; noticed that the attacks were also precipitated location of the internal capsule. Visual evoked 45:67-72. sudden unexpected potentials and lower extremity somato- by a noise or the appearance of a cyclist or a car. She first sensory evoked potentials showed prolonged sought medical attention five months later wave latencies. CSF showed an elevated when she developed urgency of micturition protein (63 mg/dL) and myelin basic protein and a diminution of dexterity of her right (14 8 mg/mL), and five oligoclonal bands. The patient was treated with a 10 day course hand. The attacks now included slurred Hemiageusia: an unusual presentation speech and rotational posturing of the head, of IV methylprednisone and his hemiparesis of multiple sclerosis recovered. On examination he still had the right arm and leg lasting a few seconds and occurring five to 10 times daily. The attacks previously described deficits. Patients with multiple sclerosis (MS) rarely Hemiageusia involving the entire right half were provoked by emotion, acceleration of complain of taste disturbances, although of the tongue is usually explained by a lesion movement, speaking and writing. Neuro- electrogustatory examinations often demon- in the ipsilateral nucleus solitarius, where logical examination was normal. Two months strate dysfunction of the taste pathway in fibres from the lingual nerve (anterior two later she was occasionally able to avert the patients with advanced disease, especially in thirds of the tongue) and fibres from the attacks by completely arresting her move- those with prominent brainstem involve- glossopharyngeal (posterior third) ments as soon as prodromal symptoms oc- nerve ment. come together.2 Recent evidence suggests the curred. The attacks occurred now five to 10 A 25 year old native American man presen- of taste pathway times per hour and lasted from about five to presence an accessory ted with a two day history of gradually through the trigeminal nerve.3 Hypogeusia is fifty seconds. progressive loss of taste on the entire right found in 5-10% of patients with advanced Neurological examination revealed inter- half of his tongue (hemiageusia). One week MS' and is frequently associated with sensory nuclear ophthalmoplegia, cerebellar gait, later he developed numbness of the right dystonia of the right leg and hypertonic involvement of the trigeminal nerve.5 Taste inner cheek, double vision, and a tendency to hemiparesis of the right side. Cerebrospinal disturbance as the initial symptom of MS has fall to the left. He had clockwise rotatory fluid contained 26 mononuclear leukocytes, a previously been reported only by Harris.5 His nystagmus, right internuclear ophthal- slightly increased protein content with an patient, a 21 year old woman, developed moplegia, left central facial palsy, left increased IgG level and non-specific multiple numbness of the right side of her face and hyperreflexia, and intention tremor with the right sided hemiageusia, that persisted for oligoclonal bands in the alkaline region at iso- right hand. The right hemiageusia was one year. Four years later she presented with electric focussing. CT scan of the head unchanged. The gag reflex was diminished on showed a paraventricular hypodense area in trigeminal neuralgia, but it was not until 10 the right side, and the right palate and the the region of the caudate nucleus. MRI years later, that multifocal symptoms led to right inner cheek were numb. Routine the diagnosis of MS.' The remarkable aspect revealed in the proton density images and the laboratory tests were all normal. CSF analysis of our case is that right-sided hemiageusia T, weighted pictures high signal emitting revealed no red cells, five lymphocytes, was the sole presenting symptom, although lesions paraventricular, in the putamen and 53 mg/dL protein, normal glucose, 5-6 ng/ investigation demonstrated multifocal the mL myelin basic protein (normal range 0-5 1 lesions. Right central nervous system ng/mL), and three oligoclonal bands without involvement occurred trigeminal sensory correlates in serum. almost two weeks after the hemiageusia, and Eleven days after the onset of the taste widespread brainstem prominent, more disturbance he developed paroxysms of pain symptomatology developed only later. around the right eye, perioral numbness and ALVARO PASCUAL-LEONE tingling sensation in the right cheek. New IRFAN ALTAFULLAH ANIL DHUNA Department of Neurology, University of Minnesota and Hennepin County Medical Center, Minneapolis, MN, USA 1 Rollin H. Geschmackstorungen bei multipler Sklerose. Laryng Rhinol 1976,55:678. 2 Carpenter MB. Human neuroanatomy. 7th ed. Baltimore: Williams and Wilkins, 1976. 3 Grant R, Ferguson MM, Strang R, Turners JW, Bone I. Evoked taste thresholds in a normal population and the application of electro- gustometry to trigeminal nerve disease. J Neurol Neurosurg Psychiat 1987;50:12. 4 Spillane JD, Wells CEC. Isolated trigeminal neuropathy. Brain 1959;82:391. Figure Transverse section through the lateral 5 Lecky BRF, Hughes RAC, Murray NMF. ventricle with T2 weighted images of the MRI Trigeminal sensory neuropathy: a study of 22 demonstrates high signal emissions in the right cases. Brain 1987;110:1463. globus pallidus and thalamus and in the Figure T2-weighted axial MRI scan (1 5 6 Harris W. Rare forms of paroxysmal trigeminal subcortical paraventricular regions in both tesla}. Increased signal noted by the floor of the neuralgia, and their relation to disseminated hemispheres. sclerosis. BMJ 1950;2:1015. fourth ventricle.
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