Letters to the Editor

Correspondence to: Dr J Berciano, Service ofNeurology, University Hospital "Marques deValdecilla", 39008 Santander, Spain.

1 Ramos A, Quintana F, Diez C, Leno C,Berciano J. CT findings in spinocerebellardegeneration. AJNR Am J Neuroradiol 1987;8:635-40.

2 Berciano J, Diez C, Polo JM, Pascual J, FigolsJ. CT appearance of panencephalopathic andataxic type of Creutzfeldt-Jakob disease. JComputAssist Tomogr 1991;15:332-34.

3 Oganda T, Inugami A, Fujita H, et al. Serialpositron emission tomography with fluo-deoxyglucose F 18 in Creutzfeldt-Jakob dis-ease. AJNR Am J Neuroradiol 1995;16:978-81.

4 Tokuomi H, Uchino M, Imamura S,Yamanaga H, Nakanishi R, Ideta T.Minamata disease (organic mercury poison-ing): neuroradiologic and electrophysiologicstudies. Neurology 1982;32:1369-75.

5 Mammack JE, Kimmel DW, O'Neill BP,Lennon VA. Paraneoplastic cerebellardegeneration: a clinical comparison ofpatients with and without Purkinje cell cyto-plasmic antibodies. Mayo Clin Proc 1990;65:1423-31.

Ferreol-Besnier disease with associatedrecurrent meningitis

We report an unusual case of a recurrentcutaneous syndrome regularily associatedwith a lymphomonocytary meningitis.A 40 year old white man presented with a

complaint of throat pain and a burning sen-sation on the inside of the hands and feetstarting three days previously, followed a daylater by fever and severe headache.

Neurological examination was unremark-able except for meningism. On the inside ofboth hands and feet extensive reddening andearly desquamation were noted. Examina-tion of CSF showed normal protein, lactate,and sugar content, no local IgG synthesis,and 216 ,ul, 80% of which were lymphocytesand monocytes with a high proportion oflarge, fragile endothelial cells; 20% werepolynuclear cells and about half of thesewere eosinophils. No infectious agent,including hepatitis B and herpes simplexviruses, was demonstrated serologically, inculture or by virus isolation. Antibody stud-ies for connective tissue disease, T cell

Patient's rightfoot on sixth day after admission,showing extensive desquamation.

differential count, streptolysin titres, com-plement levels, and electrophoresis forimmunoglobulin were normal.

Fever and headache subsided within fourdays. Two days later, extensive desquama-tion with almost complete shedding of pal-mar and plantar skin occurred (figure). Thepatient had had six similar attacks since1986 with intervals from seven to 18months. All episodes started with throatpain and a burning sensation on palmar andplantar skin, followed by fever and headachethat subsided within days, accompanied byextensive desquamation of affected skin, ontwo occasions also of several nails.Neurological abnormalities other thanmeningism were not found. Brain CT andMRI were normal on the several occasionsthat they were done. No leak was demon-strated on CSF scintigraphy. During fiveepisodes a lymphocytic CSF pleocytosis wasdocumented. No infectious agent was iso-lated. On two occasions a slight transientproteinuria was noted. The patient was wellbetween attacks, had no skin abnormalitiesin the intervals, and did not require regularmedication.The cutaneous syndrome conforms to the

rare clinical entity of erythema scarlatini-forme desquamativum recidivans of Ferreol-Besnier. It is characterised by recurrentattacks of a prodromal phase with head andmuscle aches, gastrointestinal and enteriticsyndromes, and fever, followed by a macularerythema leading to the pathognomonicdesquamation and scalding of palmar andplantar skin.' Patients are symptom free dur-ing the intervals, which may last from weeksto several years. About 40 definite caseshave been reported since the disease wasdescribed in 1878. Localised variants inwhich only the hands and feet are involvedcorrespond precisely to the cutaneous syn-drome found here.' Throat pain and tran-sient proteinuria, unusual for recurrentmeningitis, are common. The aetiology isunknown, but abnormal cutaneous reactionto an infectious disease has been proposedas the cause in some cases.' No infectiousagent was isolated. Connective tissue dis-eases, immunosuppression, and uveomenin-gitic syndromes were excluded by clinicalpresentation and appropriate laboratorystudies.

Interestingly, the CSF cytology, with amixed lymphomonocytary pleocytosis withlarge fragile endothelial cells, conforms tothe picture seen in benign recurrent asepticmeningitis (Mollaret's meningitis).2 Anunusual feature was the high number ofeosinophils, rarely reported in this disease.3The clear association in this case of

Ferreol-Besnier disease with recurrent CSFlymphocytosis has no precedent in the litera-ture. However, we located two patients pre-senting with an urticarial rash duringepisodes of recurrent meningitis. In thesecases possible aetiologies were lymphoma4and familial Mediterranean fever.5 Thesediagnoses should be borne in mind whenconfronting a patient with the rare picture ofrecurrent meningitis associated with cuta-neous symptoms.

We thank Dr Niedecken, Dermatology Service,State Clinic of the Rhine, Bonn, for helpful discus-sion.

ANDREAS MEYER-LINDENBERGCentre for Psychiatry, J7ustus-Liebig-University

Medical School, Gie,ien, GermanyMICHAEL HOTZ

Department ofNeurology, State Clinic of the Rhine,Bonn, Germany

Correspondence to: Dr Andreas Meyer-Lindenberg, Zentrum fur Psychiatrie, Justus-Liebig-Universitat GieBen, Am Steg 22, 35385Giei3en, Germany.

1 Landthaler M, Michalopoulos M, Schwab U,Dorm M. Erythema scarlatiniforme desqua-mativum recidivans localisatum. Hautarzt1985;36;581-5.

2 Frederiks JAM, Bruyn GW. Mollaret's menin-gitis. In: McKendall RR, ed. Handbook ofclinical neurology, Vol 12 (56): viral disease.Amsterdam: Elsevier, 1989;627-35.

3 Bamborschke S, Sandmann J, Wullen T. Diebenigne rezidivierende aseptische Meningitisnach Mollaret. Kasuistik, liquorzytologischeBefunde und Literaturubersicht. (Mollaretbenign recurrent aseptic meningitis. Casereport, results of cerebrospinal fluid cytologyand review of the literature.) Nervenarzt1990:61;615-9.

4 Swithinbank IM, Rake MO. A case ofMollaret's meningitis associated with a lym-phoma. Postgrad MedJ 1978;54;682-5.

5 George RB, Westphal RE. Periodic meningitis.An unusual manifestation of periodic dis-ease. Ann Intern Med 1965;62;778-81.

Trigeminal neuralgia in pontineischaemnia

Trigeminal neuralgia occurs in several con-ditions involving slight damage of thetrigeminal root entry zone into the pons.' Toour knowledge there are no reported cases oftrigeminal neuralgia occurring after brain-stem ischaemia. We report one such patient.A 58 year old man had had trigeminal

neuralgia in the territory of the secondbranch of the right trigeminal nerve for fouryears. Carbamazepine (200 mg twice daily)had been effective for the first two to threeyears of his pain, but was useless when wefirst saw him. Neurological examinationshowed slightly diminished superficial sensa-tion in the territory of the second and thirdbranch of the right trigeminal nerve, andwas otherwise normal. Comeal reflex wasnormal bilaterally. The sensory loss was con-firmed by quantitative sensory testing.2Trigeminal evoked potentials (TEPs)3 wereobtained after stimulation of the infraorbitalnerve. On the left side they were normal,

T2 weighted axial MRI. Patient's right side ison the figure's left hand side. A hyperintensespot-like area (arrow) is present in the rightlateral part of the pons, corresponding to thetrigeminal root entry zone. This area was notseen in corresponding Tl weighted images, butwas seen in proton-density images (not shown).These findings are indicative ofa smallischaemic lacune.

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Letters to the Editor

whereas on the right side all componentsbeyond W2 were delayed and reduced inamplitude, suggesting an impairment ofafferent conduction at some site between thetrigeminal root and the trigeminal nuclei.4Increasing carbamazeine dosage to 400 mgthree times daily caused the pain to disap-pear. Two years later his symptoms wors-ened. Neurological examination and TEPswere unchanged. Magnetic resonanceangiography showed multiple ischaemiclesions in the cerebral hemispheres withwidespread cortical atrophy. An ischaemiclesion was found in the right lateral part ofthe pons, in the trigeminal root entry zone(figure). Multiple sclerosis and Lyme diseasewere ruled out by the clinical history and byappropriate investigations. Radiofrequencyselective thermal rhizotomy was followed bya slight, further decrease of tactile and painsensation in the right trigeminal territory(second branch) and by disappearance ofthe pain.

In this patient the typical pain of trigemi-nal neuralgia was associated with anischaemic lesion strictly localised to the ipsi-lateral trigeminal root entry zone. This asso-ciation may have been, in theory,coincidental, but it seems unlikely. In fact,alterations in TEP showed a functionaldamage of the afferent pathway at the samesite where altered morphology was detectedby MRI. Furthermore, the ischaemic lesionwas exactly at the trigeminal root entry zone,an area where most lesions causing sec-ondary trigeminal neuralgia are located.'Neuralgic pain waxed and waned for manyyears: this is expected, as trigeminal neural-gia is known to show remissions and recur-rences secondary to permanent trigeminallesions.' We recommend that brainstemischaemia is included in the differential diag-nosis of trigeminal neuralgia, especiallywhen the neurological examination disclosesalterations in trigeminal nerve function.Measurement of TEP is useful in the searchfor such alterations. In our patient the painwas successfully treated with carbamazepinefor several years, and was eventually relievedby radiofrequency selective thermal rhizo-tomy, a procedure that may not necessarilybe confined to the treatment of "essential"trigeminal neuralgia.

MAURIZIO BALESTRINOMASSIMO LEANDRI

Department ofNeurological Sciences, University ofGenova, Italy

Correspondence to: Dr M Balestrino,Dipartimento di Scienze Neurologiche, Via DeToni 5, 16132 Genova, Italy.

1 Selby G. Diseases of the fifth cranial nerve. In:Dick PJ, Thomas PK, Lambert EH, BungeE, eds. Penpheral neuropathy. 2nd ed. Vol II.Philadelphia: WB Saunders, 1984:1224-65.

2 Fruhstorfer H, Lindblom U, Schmidt WG:Method for quantitative estimation ofthermal thresholds in patients. J NeurolNeurosurg Psychiatry 1976;39:1071-5.

3 Leandri M, Parodi CI, Favale E. Normativedata on scalp responses evoked by infraor-bital nerve stimulation. ElectroencephalogrClin Neurophysiol 1988;71:415-21.

4 Leandri M, Campbell JA. Origin of early wavesevoked by infraorbital nerve stimulation inman. Electroencephalogr Clin Neurophysiol1986;65: 13-9.

MRI demonstration of reversibleimpairment of the blood-CNS barrierfunction in subacute combined degener-ation of the spinal cord

We report clinical, laboratory, and imagingfindings in a case of subacute combineddegeneration of the spinal cord. The 50 year

(A) Sagittal Tl weighted image after (B) Sagittal Tlweighted image afteradminstration of 15 ml contrast agent. Apart administration of 15 ml of contrast agent. Afrom larger lesions at the thoracic and lower nodular contrast enhancing lesion is present incervical level (large arrows), many smaller the posterior cervical cord (arrow).pearl-like contrast enhancing spots can be seen(small arrows).

old woman presented with tickling sensa-tions running down her back when bendingthe head. Five months before she had firstnoticed numbness of her feet which slowlyascended to the level of her nipples. Duringthe past six months she had lost 7 kg inweight.

Forward flexion of her neck inducedLhermitte' s phenomenon. The tendonreflexes of her legs and plantar responseswere absent. Complete loss of light touches,vibration, and position sense was foundbelow D5. Pain and thermal perceptionwere not diminished. Her gait was unsteadydue to a sensory ataxia.Her mean red cell volume was 110 fl and

serum vitamin B12 concentration was44 pmol/l. Haemoglobin, packed cell vol-ume, and folate were normal. The two stage"Schilling test" showed intestinal malab-sorption of vitamin B 12, not due to lack ofintrinsic factor. Gastric endoscopy was non-specific. analysis of CSF was normal.

Somatosensory evoked potentials fromtibial and sural nerves showed abnormalitiesin latency indicative of a lesion in the poste-rior columns of the spinal cord; thesomatosensory evoked potentials from bothmedian nerves were normal. Nerve conduc-tion studies of the sural nerves disclosed areduced nerve conduction velocity indicativeof a demyelinating neuropathy.Two weeks after treatment with 1000 ,g

vitamin B12 daily, there was an almost com-plete restitution of sensory functions.

T2 weighted MRI images of the thoracicspinal cord showed an ill defined hyperin-tense lesion in the posterior parts of thespinal cord. Ti weighted images afteradministration of gadolinium DTPA showedmultiple slightly expansive, contrast enhanc-ing lesions in the posterior column of thecervical and thoracic spinal cord (fig A, B).After 18 days of treatment the lesions haddisappeared.

In summary, our patient had the classicclinical signs of spinal cord degenerationincluding Lhermitte's phenomenon, sensoryimpairment, dysaesthesia, and sensory ataxiaof the lower limbs. In all cases reported sofar, MRI studies were performed in spinalcord degeneration to exclude a spinal cordcompression."'- The lesions detected ashyperintensities in T2 weighted images werealways located in the posterior columns ofthe spinal cord. The thoracic region wasaffected in all patients. Signal abnormalitiesin the cervical region were seen in twocases.4 5

We found a very pronounced, multifocalcontrast enhancement of the cervical andthoracic sections of the spinal cord indicat-ing blood-CNS barrier disruption. Thelesions were multifocal and located close tothe spinal cord surface. A slight degree ofexpansion was noted. Thus a granulomatousinflammation, multiple sclerosis lesions, ortumour metastases had to be considered.The clue to spinal cord degeneration in thiscase was the location of the lesions exclu-

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