1122 Letters to the Editor J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.12.1122 on 1 December 1991. Downloaded from sitivity.? We present a case of OMM without pseudobulbar palsy. The association of vitro.8 Whipple's disease is associated with symptoms of Whipple's disease and with supranuclear gaze paresis with oculomas- immunodeficiency.8 Thus intestinal wall negative peroral jejunoileal biopsies, which ticatory myorhythmia, however, seems to be macrophages are ineffective in phagocytosing indicates the usefulness of laparotomy for pathognomonic of the effects of Whipple's intracellular gram positive bacilli, resulting in jejunoileal biopsies as an alternative to brain disease on the CNS.'2 This led us to perform inability to eliminate chronic infection.9 This biopsy to confirm Whipple's disease. surgical jejunal and mesenteric lymph node suggests that Whipple's disease may be con- A 47 year old woman was admitted in May biopsies rather than a brain biopsy despite sidered as a disease of macrophages.8 The 1988 in a depressed state. In June 1987 she negative endoscopic and numerous peroral periventricular and periaqueductal distribu- had noted progressive visual disturbance. distal jejunal and ileal biopsies. The nor- tion of the CNS involvement in Whipple's Rhythmic elevations of her right upper lip mality ofthe CT and MRI scans also suppor- disease consists of macrophagic infiltration appeared, and later, paroxysmal hypersomnia ted this decision. and subependymal nodules. Such a and considerable weight gain (10 kg). A nine The possibility of brain involvement with- "tumoural" involvement may explain why month course oftreatment for depression was out systemic manifestation in Whipple's dis- antibiotics with good BBB diffusion are not ineffective and her symptoms and signs ease should be kept in mind. This necessitates effective in this CNS disease. progressively deteriorated. Examination on a search for the disease by endoscopic and PIERRE AMARENCO ETIENNE ROULLET admission showed complete vertical and peroral jejunoileal biopsies in all patients with LAURENT HANNOUN horizontal ophthalmoplegia with sparing of unexplained supranuclear oculomotor palsy. RENE MARTEAU oculocephalic reflexes (which appeared spon- Despite two single reports of reversible CNS Service de Neurologie et Service de Chirurgie, the doll's eyes phenomenon the treatment of Hbpital Saint-Antoine taneously, giving involvement,4 5 however, 184, rue du Fg St-Antoine, with all movements of the head). Her eyes, CNS Whipple's disease has so far been dis- 75571 Paris Cedex 12, France. part of her limbs were mild in face, and proximal appointing.6 The improvement Correspondence to: Dr Amarenco, Service de affected by myorhythmia, which consisted of neurological states on pefloxacine was impor- Neurologie, Hopital Saint-Antoine. masticatory movements with synchronous tant in our patient but needs further confir- adduction of her eyes (convergence) and is a quinolone which mation. Pefloxacine 1 Schwartz MA, Selhorst JB, Ochs AL, et al. limbs at about 1 cps. Her soft palate was not readily diffuses across the blood-brain barrier Oculomasticatory myorhythmia: a unique involved. Movements also occurred during (BBB) and is effective against intracellular movement disorder occurring in Whipple's sleep. Gait disturbance and a slight facial micro-organisms such as those supposed to disease. Ann Neuroll986;20:677-83. Her 2 Hausser-Hauw C, Roullet E, Robert R, Marteau akinesia was noted but no neck rigidity. be the cause of Whipple's disease. Neverthe- R. Oculo-facio-skeletal myorhythmia as a mental state was normal. There was a less, taking into account the specificity of cerebral complication of systemic Whipple's pseudobulbar type dysarthria but no OMM in the diagnosis of Whipple's disease disease. Mov Disord 1988;3:179-84. difficulty in swallowing. No other systemic the of treatment6 were we 3 Adams M, Rhyner PA, Day J, DeArmond S, and poor efficacy Smuckler EA. Whipple's disease confined to sign or symptom was recorded; there was no justified in performing a laparotomy to con- the central nervous system. Ann Neurol diarrhoea. Her CSF was normal with no PAS firm the diagnosis? We think that this condi- 1987;21:104-8. positive cells present. EEG, CT, and Tl- tion needs to be formally recognised and 4 Ryser RJ, Locksley RM, Eng SC, Dobbins WO, on such a Schoenknecht FD, Rubin CE. Reversal of weighted (DTPA-Gadolinium enhanced) confirmed before embarking long dementia associated with Whipple's disease and T2-weighted MRI scans yielded normal and difficult treatment. by trimethoprim-sulfamethoxazole, drugs results, as did endoscopy and 30 peroral distal Riggs has suggested that incidental use of that penetrate the Blood-brain barrier. Gas- and proximal jejunal and ileal biopsies. may eradicate gastrointestinal troenterology 1984;86:745-52. antibiotics 5 Alder CH, Galetta SL. Oculo-facial-skeletal Erythrocyte sedimentation rate was 35 mm involvement in presymptomatic Whipple's myorhythmia in Whipple Disease: Treatment per hour. Schilling's test was 5% (normal disease but not the meningeal involvement with ceftriaxone. Ann Intern Med range 12-30%), suggesting distal ileal because of their poor BBB diffusion.' This 1990;1 12:467-9. isolated and late 6 Keinath RD, Merrell DE, Vlietstra R, Dobbins involvement. may explain the apparently WO. Antibiotic treatment and relapse in A laparotomy was performed and surgical CNS involvement. Such a hypothesis con- Whipple's disease. Gastroenterology 1985; biopsy specimens of the jejunum and mesen- siders only the infectious mechanism and 88:1867-73. nodes were taken. Pathological not the poor effect of some 7 Riggs JE. The evolving natural history of teric lymph does explain neurologic involvement in Whipple's disease: examination showed no jejunal abnormality antibiotics which are well able to cross the a hypothesis. Arch Neurol 1988;45:830. but PAS positive cells with positive Gram BBB. Unlike most infectious diseases, there 8 Dobbins WO. Whipple's disease. In: Berk JD stain in the mesenteric lymph nodes were are no established cases ofdirect transmission ed. Gastroenterology. Philadelphia: WB Saun- to ders Company, 1985:1803-13. found, confirming Whipple's disease. Treat- of Whipple's disease from one patient and gastroin- 9 Webster ADB. Immunodeficiency http://jnnp.bmj.com/ ment with rifampicin and tetracycline for two another, no reproduction of the disease in testinal disease. In: Triger DR ed. Clinical months stabilised the disease. Trimetho- laboratory animals, and no convincingly immunology of the liver and gastrointestinal prim-sulfamethoxazole and chloamphenicol specific organisms isolated by culture in tract. Bristol: Wright, 1986:127-49. were then given for a further two months. The results of Schilling's test returned to normal but signs and symptoms did not improve. Chloramphenicol had to be with- Binswanger's clinical and neuropatho- their suggestions on current English and drawn and was replaced by pefloxacine. logical criteria for "Binswanger's dis- French literature. It might be of interest to Two and a half years after the onset of ease" compare these modern criteria to Binswan- treatment there was mild improvement of ger's original description,2 which had only on September 28, 2021 by guest. Protected copyright. signs. Masticatory movements remained the Bennett et al' have made a timely and critical been available in English in a grossly trun- same, but the adduction of the eyes had attempt to standardise the diagnostic criteria cated form.3 disappeared; the eyes could be displaced to for "Binswanger's disease". They have based The table shows the clinical hallmarks the left and right to 50% and slightly down- Table Binswanger's clinical and neuropathological criteriafor the diagnosis of "Encephalitis ward but not upward. Unsteadiness of gait, subcorticalis chronica progressiva"2 probably due to the supranuclear oculomotor palsy, persisted. Her mental state was largely Clinical criteria: (verbatimfrom reference 2, page 1184) status was unaffected (minimental 30/30). the disease begins at the onset of senility (early in the fifties) or in advanced old age (early in the sixties); Her full scale intellectual quotient on the slow impairment of intellectual capabilities mainfesting primarily by the progressive impairment and WAIS fell slightly from 92 to 86 then in- ultimate loss of the association between cortical sensory and motor areas; creased to 96 in 1990. An uncontrolled new most frequently observed are aphasic disturbances (as in the present case), hemiamblyopia or hemianopia, hemiparesis with loss of the sense of pressure, position or touch; synchronous movement of flexion extension these circumscribed deficits are of a stable character during the fully developed disease and they are of the right thigh appeared when she relaxed combined with the slow and relentless deterioration of intellectual performance; in bed. Worsening of hypersomnia and ap- .... until) the patients resemble decerebrate laboratory animals. doses of pearance of cataplexy required high criteria: (from reference 2, page 1137) imipramine which gave partial relief. Neuropathological a we find a pronounced atrophy of the hemispheric white matter, either restricted to one or more gyri in Progressive supranuclear palsy is feature one brain area or of several hemispheric regions affected with variable severity; of Steele Richardson Olszewski disease, a these changes are most clearly found in the area of the occipital and temporal lobes, so that the temporal degenerative disorder of unknown aetiology and occipital horn are widened into bag-like cavities, no known treatment. A supra- while the anterior portion of the lateral ventricle shows relatively little enlargement and the frontal white and with matter is almost unaffected by the disease process. nuclear oculomotor paresis for downgaze is The cortex does not show any remarkable macroscopic change apart from a slight narrowing. one of the main characteristics of this syn- Invariably, these cases show severe atheroma of the cerebral arteries. drome, together with axial rigidity and Letters to the Editor 1123 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.54.12.1122 on 1 December 1991. Downloaded from and the neuropathological findings which to changes in posture. The blurring became Our patient stopped using lindane when Binswanger considered essential to a disease persistent after three months and then he the association of pseudotumour cerebri and which he called "encephalitis subcorticalis developed intermittent blurring in his left lindane was brought to his attention; this was chronica progressiva". These "reliable eye. Shortly after he noticed early morning coincidentally reinforced when a neighbour's criteria" were introduced to differentiate occipital headaches and tinnitus. He had used puppies convulsed and died after exposure to "encephalitis subcorticalis" from "arterio- benzene hexachloride at least twice a month a 20% solution. Despite discontinuation of sclerotic brain degeneration" (which also for about 30 years to rid his beagle hounds of the pesticide the patient's intracranial pres- affects the cortex), from the "general paralysis fleas and ticks. He said he used a 20% sure remained elevated (440 mm CSF) and of the insane" and from senile dementia, concentrate to make dip and spraying headaches continued 11 months later when a which he knew could also be accompanied by applications but wore a mask and appropriate lumbo-peritoneal shunt was inserted. white matter changes.2 protective clothing. He was well built but not Removal of the toxin should result in allevia- The similarities between Bennett's' and obese. His neurological examination yielded tion of increased intracranial pressure. Per- Binswanger's own criteria are obvious. normal results except for visual findings: his haps the lindane caused permanent or prolon- Nevertheless, several striking discrepancies best corrected visual acuity was 6/36 OD and ged alteration of the arachnoid villi. Alter- appear noteworthy. The white matter atro- 6/9 OS. He had a right relative afferent natively, lindane may be present in fat cells phy in Binswanger's patients was most pro- pupillary defect. Ocular motility and slit lamp for an extended period and have a long lasting nounced in the occipital and temporal lobes, examinations were normal. Intraocular pres- effect on CSF absorption. Whether the whereas radiological changes are most com- sures were 21 and 23 mm Hg respectively. patient's liver damage was caused by previous monly found in the frontal lobes. According Ophthalmoscopic examination showed dis- chronic alcohol consumption or exposure to to Binswanger, "encephalitis subcorticalis" tinct swollen optic discs with small cups, loss lindane is unclear. The relation with lindane slowly and relentlessly progressed to a state of the nerve fibre layer in the right eye, and a exposure may not be coincidental because of decerebration, whereas Bennett et al ex- small pseudodrusen in the left eye, typical of other pesticides have been linked to cluded patients with severe dementia. Bins- chronic papilloedema. Goldmann perimetry pseudotumour cerebri in the past.2 The use wanger assumed that arteriosclerosis was the showed visual field loss characteristic of of lindane should be discontinued when cause ofdisease and mentioned the invariable chronic papilloedema. patients have unexplained raised intracranial presence of cerebral arteriosclerosis (which MRI of the head was normal except for a pressure. was, however, very mild in the only case he few small periventricular white matter LISA VERDERBER described extensively). He did not describe lesions; venous sinus thrombosis was not PATRICK LAVIN or other evidence of RALPH WESLEY hypertension systemic seen. A spinal tap showed an opening pres- Departments ofNeurology and Ophthalmology, vascular disease. sure of 400 mm CSF with one monocyte per Vanderbilt University Medical Center, It has already been pointed out that the cu mm, protein 0-34 Gm/l, glucose 2 mmol/l, Nashville, TN 37212, USA. relationship between Binswanger's findings and no evidence of infection. Other Correspondence to: Dr Lavin, M.D., 2100 Pierce and the modem "Binswanger's disease" laboratory values were notable only for Ave., Nashville, TN 37212, USA. remains open to question.' Binswanger did elevated cholesterol and triglyceride concen- not present a full account of the histopath- trations and mildly abnormal results of liver ology in his cases. This was left to Alzheimer, function tests. Thyroid function tests were 1 Ireland B, Corbett JJ, Wallace B. The search of who first used the eponym "Binswanger's normal; rheumatoid factor and antinuclear causes ofidiopathic intracranial hypertension. disease",4 and to Nissl.' Inconsistencies in antibodies were negative. Toxic screens for Arch Neurol 1990;47:315-20. Binswanger's original description may sup- lead, mercury, and arsenic were negative. 2 Sanbom GE, Selhorst JB, Calabrese VP, et al. port the speculation that he eventually re- Management included Pseudotumour cerebri and insecticide intox- dietary advice (weight ication. Neurology 1979;29:1222-7. garded the differentiation of such vascular loss), diuretics, and prednisone, but he sub- 3 Pramanik AK, Hansen RC. Transcutaneous dementias as too difficult or too unrewarding.2 sequently had bilateral optic nerve sheath gamma benzene hexachloride absorption and HANS FORSTL fenestration because of progressive visual toxicity in infants and children. Arch Der- ROBERT HOWARD matol 1979;115:1224-5. RAYMOND LEVY field loss. Ten months after diagnosis his field 4 Lee B, Groth P. Scabies: transcutaneous poison- Section of Old Age Institute ofPsychiatry, defects were stable, but his visual acuity ing during treatment. Pediatrics 1979;59:643. London SE5 8AF, UK remained impaired. 5 Solomen LM, Fahrner L, West DP, et al. Lindane, a gamma isomer of hexachloro- Gamma benzene hexachloride toxicity. Arch 1 Bennett DA, Wilson RS, Gilley DW, Fox JH. Dernat 1977;113:353-7. Clinical diagnosis of Binswanger's disease. cyclohexane used as a pesticide and an 6 Danopoulos E, Melissinos K, Katsas G. Serious Neurol ectoparasiticide, is metabolised by the liver poisoning by hexachlorocyclohexene. Clinical J Neurosurg Psychiatry 1990;53: http://jnnp.bmj.com/ 961-5. and distributed and stored in depot fat and and laboratory observations on 5 cases. Arch 2 Binswanger 0. Die Abgrenzung der allgemeinen of Indust Hygien 1953;8:582-7. progressiven Paralyse, I-III. Berl Klin other lipophilic tissues. It is commonly pres- 7 Heuser M, Heuser H. Pseudotumour cerebri Wochenschr 1894;49:1103-5, 1137-9, 1180-6. cribed topically as a 1% solution for scabies following insecticide (lindane) intoxication. (Tranl. by H Forstd et al: Binswanger on but is available in concentrations of 0 5%- Acta Univ Carol Med. 1976;75:133-5. Binswanger's disease. Intern J Geriatr Psy- 99%; our patient used a 20% veterinary 8 Sufnol C, Tussell JM, Gelpi C, Rodriguez-Farre chiat 1991;6:529-35.) E. Convulsant effect of lindane and regional 3 Olszewski I. Subcortical arteriosclerotic ence- concentration for his dogs. Lindane is a brain concentration of GABA and dopamine. phalopathy: review of the literature on the so- powerful CNS stimulant known to cause Toxicology 1988;49:247-52. called Binswanger's disease and presentation headache, nausea, vomiting, diarrhoea, con- 9 St Omer V. Investigation into mechanisms res- of 2 cases. World Neurol 1962;3:359-75. ponsible for seizures induced by chlorinated 4 Alzheimer A. Die Seelenstrorungen auf vulsions, muscle spasms, respiratory failure hydrocarbon pesticides. J Neurochem 1971; on September 28, 2021 by guest. Protected copyright. arteriosklerotischer Grundlage. Allg Z Psy- with cyanosis, coma, and death.'5 "Optic 18:365-74. chiat 1902;59:695-701. (Transl: Mental dis- neuritis" after "improper use" of lindane turbances ofvascular origin.) Neuropsychiatry powder has also been reported.6 Heuser and Neuropsychol Behav Neurol (in print). 5 Nissl F. Zur Kasuistik der arteriosklerotischen Heuser briefly described "pseudotumour Demenz (ein Fall von sogenannter Ence- cerebri" in a farmer with localised brain phalitis subcorticalis). Z Ges Neurol Psychiat oedema, after "prolonged professional 1920;19:438-43. inhalation" oflindane;7 but the appearance of the optic discs and the spinal fluid pressure Motor neuron syndrome in the arms were not reported. The mechanism oflindane after radiation treatment toxicity is unknown, although it is highly Pseudotumour cerebri and chronic ben- lipid soluble and may act as a gamma-amino- Radiation myelopathy is a rare but well zene hexachloride (lindane) exposure butyric acid-A (GABA-A) receptor antagon- established complication of radiotherapy ist to produce convulsive effects and inter- leading to diagnostic difficulties with Pseudotumour cerebri, the syndrome of ference with the production and utilisation of neurological complications of the primary idiopathic intracranial hypertension and free ammonia in the brain.89 Chlordecone, a neoplasm, like epiduritis or spinal metas- papilloedema in the absence of a tumour or cyclodiene insecticide which also induces tasis.' We report a rare case of radiation obstructive hydrocephalus, may be associated seizures, has been implicated in causing myelopathy presenting as a cervical motor with exposure to drugs or toxins."'' We report pseudotumour cerebri by inhibition of ATP- neuron syndrome that developed three years a patient, repeatedly exposed to the pesticide ase activity, resulting in impaired resorption after local radiotherapy in which spinal cord benzene hexachloride (lindane), who de- of cerebrospinal fluid across the arachnoid magnetic resonance imaging (MRI) showed a veloped chronic intracranial hypertension. villi.' Lindane and chlordecone may have centrospinal cystic lesion. A 45 year old man (weighing 80 kg) who similar effects on the arachnoid villi as they A 44 year old man without relevant history kept hounds noted fleeting episodes of are both lipid soluble, neurotoxic chlorinated presented with dyspnoea and a rapidly grow- blurred vision in his right eye usually related hydrocarbons. ing cervical anterior mass. We found a mal-