Ness Improved Gradually and Four Weeks Examination. Distant
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198 Letters to the Editor Generalised botulism-like syndrome right sternomastoid and left splenius capitis increased sensitivity to the toxin seems after intramuscular injections of botu- muscles and 500 units into the left mid- unlikely to be present intermittently (patient linum toxin type A: a report of two cases trapezius. The neck pain resolved and the 2) and it is possible that some of the toxin neck posture improved considerably for was inadvertently injected directly into the Botulinum toxin type A (BT/A) is com- about two months. No adverse effects were vascular capillaries. monly used nowadays in the treatment of reported. The injections were repeated every The findings reported here suggest the patients with localised muscle spasticity. two to three months using the same protocol need for regular long term monitoring of The toxin, in therapeutic doses, is consid- and dosage schedule each time. One week patients treated with BT/A. The absence of ered to be effective and safe.' Systemic after the third treatment session the patient adverse effects at the commencement of adverse effects are rare and include flu-like developed transient dysphagia lasting 10 treatment should not be relied on as evi- symptoms, anaphylactic reactions, and days. There were no further adverse effects dence that a future injection would not excessive fatigue. Generalised clinically until five years later when, three weeks after cause an adverse response. not been the injection, the patient presented with dys- A M 0 BAKHEIT detectable muscle weakness has University Rehabilitation Research Unit, reported in patients treated with BT/A. phagia, severe dysarthria, diplopia, and gen- Southampton General Hospital, Southampton, UK Similarly, changes on conventional EMG in eralised, moderately severe weakness C D WARD muscles distant from the site of the toxin involving the neck, trunk, and limb muscles. University ofNottingham, Nottingham, UK D L MCLELLAN injection have not been reported,2 although Conventional intramuscular EMG of the University of Southampton, Southampton, UK single fibre EMG abnormalities have been right quadriceps femoris, tibialis anterior, previously documented.3 We report two first dorsal interosseous, and biceps muscles Correspondence to: Dr AMO Bakheit, University confirmed widespread denervation. Rehabilitation Research Unit, Mail point 874, patients in whom treatment with therapeutic Southampton General Hospital, Southampton doses of BT/A resulted in a generalised mus- Similarly, single fibre EMG of the right SO16 6YD, UK. cle weakness with widespread EMG abnor- extensor digitorum communis was abnormal malities which were typical of botulism. with very prolonged jitter values and 1 Therapeutics and Technology Subcommittee of the American Academy of Medicine. Patient 1 was a 67 year old woman with increased blocking. Assessment: the clinical usefulness of botu- longstanding spastic paraparesis due to mul- The patient's neurological symptoms and linum toxin-A in treating neurologic disor- tiple sclerosis. Four years after diagnosis the signs gradually improved over the next ders. Neurology 1990;40:1332-6. weeks and four months later her condition 2 Olney RK, Aminoff MJ, Gelb DJ, Lowenstein patient presented with painful spasticity of DH. Neuromuscular effects distant from the her hip adductors and "scissoring' of her was similar to that before the botulinum site of botulinum neurotoxin injection. gait. These symptoms responded well to toxin injection. A repeat conventional intra- Neurology 1988;38: 1780-3 intermittent obturator nerve blocks with muscular EMG was normal. The patient 3 Lange DJ, Brin MF, Warner CL, Fahn S, Lovelace RE. Distant effects of local injec- 50% alcohol. However, she started to declined to have single fibre EMG studies. tion of botulinum toxin. Muscle Nerve develop severe spasticity of the hamstring The two patients reported here developed 1987;10:552-5. muscles for which she was referred for treat- a syndrome which resembles botulism after 4 Anderson TJ, Rivest J, Stell R, et al. Botulinum intramuscular injections of therapeutic doses toxin treatment of spasmodic torticollis. J R ment with BT/A. Soc Med 1992;85:524-9. She had moderately severe spastic para- of BT/A drawn from two different batches of 5 Konstanzer A, Ceballos-Baumann AO, paresis. Muscle tone was greatly increased in the drug. In the first patient these symptoms Dressnandt J, Conard B. Botulinum toxin A the hamstring muscles of the left leg. occurred after only one dose, whereas in the treatment in spasticity of arm and leg. Mov However, the patient was able to stand inde- second they developed after five years of reg- Disord 1992;7(suppl 1):137. pendently and walk indoors with a gutter ular treatment with the toxin. Clinically frame rolater, although she could not fully detectable weakness was present in the Feeling cold: an unusual brain injury extend her left knee in stance. extraocular, bulbar, trunk, and limb muscles symptom and its treatment with vaso- The patient was treated with a total of and EMG changes were recorded in all mus- pressin 250 units BT/A Dysport which was divided cles tested. The muscle weakness and EMG between the medial and lateral hamstring changes resolved a few weeks later. Some 12 years ago, a man was assessed for muscles of the left leg. Four days after the Generalised muscle weakness has not medicolegal purposes for the late effects of a injections she complained of sudden onset of been reported previously in patients treated severe head injury sustained almost five hoarseness of her voice, inability to walk, with BT/A. Anderson et al4 have described a years earlier. After severe visual impairments and to hold her head up. Neurological patient with longstanding paralytic polio from injury to the optic chiasma, he rated as examination confirmed severe flaccid para- who reported deterioration in pre-existing his second worst problem the fact that ever plegia, severe weakness of neck flexors, dys- lower limb muscle weakness after the injec- since the injury he had always felt cold, phonia, and right partial ptosis. The rest of tion of BT/A (Botox) into his neck muscles although objectively he was no more than the physical examination was unremarkable. for spasmodic torticollis. However, muscle cool to the touch and had normal sublingual Routine investigations including a full blood weakness was not confirmed on physical temperature. He and his partner described count, erythrocyte sedimentation rate, and examination. how even in high summer he would sit in urine culture were normal. An EMG Some patients treated with BT/A had pro- front of a fire wearing two or more sweaters showed evidence of denervation in the left longed jitter values and increased blocking and a blanket or two, yet still feel uncom- hip adductors, biceps femoris and flexor on single fibre EMG recorded from muscles fortably cold. This was in stark contrast to carpi radialis. Single fibre EMG confirmed distant from the site of injection.3 This sug- his preinjury habit of driving his long dis- the increase in jitter values and blocking. gests spread of the toxin to distant muscles tance lorry in rolled up shirtsleeves with the The jitter values ranged from 92-6 to 408 ps even though the patients did not exhibit cab window open, summer and winter alike. (normal value < 57 ,us) and blocking ranged objective weakness in these muscles or have Other residual disorders were total anosmia, from 14% to 36%. abnormalities on conventional EMG. The mild brainstem motor deficits, and episodic The lower limb and neck muscle weak- co-occurrence of generalised muscle weak- dyscontrol. ness improved gradually and four weeks ness and abnormalities on conventional He also had moderately severe impair- later the findings on clinical examination EMG in our patients may be an indication ments of attention and memory. At that and functional assessment were similar to of the severity of the neuromuscular trans- time we were engaged in a formal study of those before treatment with BT/A. EMG mission block. the effects on such deficits of nasally admin- studies were not repeated. It is difficult to explain the generalised istered vasopressin (now submitted for pub- Patient 2 was a 34 year old woman with botulism-like syndrome in our patients given lication). Because the extant published multisystem atrophy who developed mild that the great majority of patients do not studies using forms of arginine vasopressin dysphagia, neck pain, and stiffness. Over the show such an effect. The total dose of the (DDAVP and DGAVP)' s were almost all next few months her chin started to progres- toxin given per session did not exceed 1000 negative, but several with lysine vaso- sively turn to the left. She had dysarthria, units of Dysport, which is well below the pressin 9 were positive, we were using the intention tremor of both hands, ataxic broad maximum recommended dose. Since the second, in the form of Syntopressin nasal based gait, a right extensor plantar response, toxin is supplied in 500 unit vials, a dispens- spray. In animal studies, this version is and spasmodic torticollis. Serum copper ing error could not have resulted in a total reported to have little vasopressor action, studies were normal and there were no acan- dose higher than 500 units in patient 1 or and, compared with the arginine derivatives, thocytes on a blood film. Brain MRI showed 1000 units in patient 2. The use by some relatively little antidiuretic but stronger brain stem and cerebellar atrophy. A trial of authors5 of up to 5000 units Dysport was mnesic effects. In keeping with the proce- anticholinergic drugs was unsuccessful. not associated with adverse effects. It is dures of the initial positive studies, a one The patient was then treated with BT/A; unlikely, therefore, that the generalised mus- month period of twice daily administration 250 units of Dysport were injected into the cle weakness resulted from an overdose.