revue neurologique 170 (2014) 228–234

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Letters to the editor Amyotrophic lateral sclerosis developing during adalimumab therapy for psoriatic arthritis Scle´rose late´rale amyotrophique au cours du traitement adalimumab de l’arthrite psoriasique

A 59-year-old male with a psoriatic arthritis history on adalimumab for eight years was referred to the Neurological Clinic of University of Athens because of progressive weakness in both lower limbs three years prior to his admission. According to British Medical Research Council (MRC) scale muscle strength was rated as 3 for iliopsoas, 3 for quadriceps, 2 for hamstrings, 3 for anterior and 3 for the posterior tibial muscles. Proximal muscles of the lower extremities were atrophic. He also had hyperactive deep tendon reflexes in lower limbs with extensor plantar response on the right side and indifferent response on the left side. Pharyngeal motor responses were absent. He presented a complete paralysis of dorsiflexion and eversion in both foot. Touch, propioception and sense of vibration were preserved. Control of bladder and bowel was normal. Mini Mental State Examination was 30. Other causes of amyotrophic lateral sclerosis ALS-like symptoms such as human immunodeficiency virus, human T-cell leukaemia virus, Lyme disease, syphilis, multiple sclerosis, post-polio syndrome, multifocal motor neuropathy, and spinal muscular atrophy were excluded. Complete laboratory tests including blood biochemistry, hemoglobin electrophoresis, serology, thyroid hormones, cerebrospinal fluid (CSF), urine analysis were within normal ranges. The patient sera was also analyzed for hexosaminidase A by a fluorometric method: Hex A–776 nmoles/h/mL (normal value: 550–1.675) and 77% of total hexosaminidase (normal value: 45–70%). IL17 serum (80.75 pg/ml) and CFS levels (32.95 pg/ml) were elevated. No serum anti-GM1 antibodies were detected. Visual examination was normal. Nerve conduction studies showed reduced amplitude of the compound muscle action potential of peroneal nerve with normal sensory and motor conduction velocities. Needle EMG detects active denervation in 3 regions. Neuroimaging of brain and spinal ruled out cord lesion. The diagnosis of ALS was made according to the modified El Escorial diagnostic criteria for ALS. We decided to discontinue patient’s treatment because his symptoms of psoriasis were almost disappeared. At the 3, 6, 9 and

12-month follow-ups, patient’s neurological and EMG examination showed mild improvement and stabilization. Patient gave his informed consent. Does psoriatic arthritis cause ALS? So far, there are no data on direct mechanism. Typical hallmarks of autoimmunity of sporadic form of ALS such as circulating immune complexes, higher frequency of a particular histocompatibility type and association with other autoimmune diseases, such as rheumatoid arthritis, lupus erythematosus and multiple sclerosis, have been reported [1–3]. The spinal cord of deceased ALS patients shows a milieu in which polarization of CD3 cells to IL-17A-producing cells can develop in response to products of macrophages, T-cells; including IL-1b, TNF-a [4]. Activation of chronic inflammation, including the IL-17A mediated pathway, is also critical in PsA, suggested to be crucial in destructive autoimmunity in both diseases [5]. This is confirmed by elevated levels of IL 17 in our patient but larger sample size is required for randomized control study. In conclusion, as far as we know this is the first report of association of psoriatic arthritis with ALS under anti-TNF therapy. Instead of a causal relationship, loss of neuronal protection by TNF-alpha activation should also be considered. More research in this field is needed for appropriate clinical decision-making.

Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.

references

[1] Pagani MR, Gonzalez LE, Uchitel OD. Autoimmunity in amyotrophic lateral sclerosis: past and present. Neurol Res Int 2011;2011:497080. [2] Rao TV, Tharakan JK, Jacob PC. Systemic lupus erythematosus presenting as amyotrophic lateral sclerosis. Clin Neuropathol 2004;23(3):99–101. [3] Trojsi F, Sagnelli A, Cirillo G, Piccirillo G, Femiano C, Izzo F, et al. Amyotrophic lateral sclerosis and multiple sclerosis overlap: a case report. Case Rep Med 2012;2012:324685. [4] Fial M, Chattopadhay M, La Cava A, Tse E, Liu G, Lourenco E, et al. IL-17A is increased in the serum and in spinal cord CD8 and mast cells of ALS patients. J Neuroinflammation 2010;7:76. [5] Raychaudhuri SP. Role of IL-17 in psoriasis and psoriatic arthritis. Clin Rev Allergy Immunol 2003;44(2):183–93.

revue neurologique 170 (2014) 228–234

A. Bougea * E. Anagnostou E. Stamboulis E. Kararizou Department of Neurology, Athens National University, Aiginition Hospital, Vass. Sofias Ave. 72–74, 11528 Athens, Greece *Corresponding author. E-mail addresses: [email protected], [email protected] (A. Bougea) Received 15 October Received in revised form 27 October Accepted 20 November Available online 18 March

2013 2013 2013 2014

0035-3787/$ – see front matter # 2014 Elsevier Masson SAS. All rights reserved. http://dx.doi.org/10.1016/j.neurol.2013.11.004

Paraneoplastic dermatomyositis with glycogen accumulation in muscle Dermatomyosite parane´oplasique avec accumulation de glycoge`ne dans le muscle

A 60-year-old woman presented facial erythema exacerbated by sun exposure. One month later, she complained of diffuse

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myalgia and arthralgia with moderate weakness, and a diagnosis of dermatomyositis was considered. Routine laboratory tests were normal, but creatine kinase level was increased (12,168 units/L; normal value 140 units/L). Needle electromyography showed myogenic patterns predominantly in girdle muscles. Body scan, upper gastrointestinal endoscopy and colonoscopy were normal. Prednisone therapy was introduced (60 mg per day), and deltoid muscle biopsy was performed 10 days after onset of corticotherapy. Paraffin sections showed variation in muscle fiber size, but neither necrosis nor inflammatory infiltrates. On frozen sections, a peripheral vacuolar appearance was consistent with glycogen accumulation in some muscle fibers. Histochemical reactions showed normal distribution of muscle fiber types, but myophosphorylase activity was deficient. Immunohistochemistry detected major histocompatibility complex class I antigen positivity on muscle fibers. Electron microscopy showed an accumulation of free glycogen in muscle fiber cytoplasm and tuboloreticular inclusions in endothelial cells (Fig. 1). One year later, the patient presented with a red-purple facial rash, eyelid swelling, and cutaneous ulceration over the index. Mobilization of wrists and finger joints was painful. She had some difficulties raising the arms and getting up from sitting, and complained of breath shortness. Chest X-ray showed a moderate bilateral pleural effusion and cardiac evaluation was in favor of a cardiomyopathy. Molecular analysis of myophosphorylase (PYGM) gene showed no sequence variations for type V glycogenosis. Biochemical studies showed moderate muscle glycogen elevation, decreased glycogenolysis and glycolysis, and reduced myophosphorylase activity. A second body scan

Fig. 1 – (A) Haematoxylin and eosin stained frozen section showing sub-sarcolemmal vacuoles in muscle fibers (at a higher magnification in inset) (scale = 100 mm). (B) Periodic acid-Schiff staining showing glycogen-filled vacuoles within muscle fibers (scale = 50 mm). Inset corresponds to myophosphorylase staining showing the complete loss of enzyme expression. (C) Immunostaining demonstrating expression of major histocompatibility complex class I antigens on muscle fibers (scale = 100 mm). (D) Electron microscopy showing a tubuloreticular inclusion in the cytoplasm of an endothelial cell (scale = 1 mm).

Amyotrophic lateral sclerosis developing during adalimumab therapy for psoriatic arthritis.

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