Salma Sakka

Clinical and electrophysiological aspects in amyotrophic lateral sclerosis syndromes associated to gammopathies

Objectives The sensory or sensitivo-motor neuropathies were the most described Peripheral neurological complications of benign or malignant monoclonal gammopathy (MG). Cases of pure motor impairment simulating amyotrophic lateral sclerosis (ALS) have more rarely been described. Herein we describe the clinical and electrophysiological aspects of three patients presenting an ALS like clinical syndrome associated to a MG and we stand out the particular features. Results There were two men and one woman with an average age of 61.3 years. All patients had a progressive weakness of 4 members and two of them presented a deglutition disorder. All patients became bedridden after few months of the onset. All patients had a very evocative clinical signs of ALS without subjective neither objective sensory troubles. An ALS clinical syndrome in its polyneuritic form was encountered in one patient. In all cases, the electromyographic data were compatible with an attack of the neurons of the anterior horn: many potentials of fibrillation, potential of fasciculation at rest and reducing of the number of potential motor unit during the voluntary contraction with increase in their amplitude and duration. Low amplitudes in motor nerve compound muscle action potentials were noted at the level of at least one nervous trunk in all patients. Low amplitudes of sensory nerve action potential were shown in one patient. MG type was demonstrated during the hospitalization in all patients. Two patients had non Ig M MG of undetermined significance and one patient had Ig M MG associated to a myeloma. Conclusion Sensory potentials impairment in ALS syndrome has to prompt us to search for an underlying aetiology and especially for GM.

Clinical presentations and electromyographical aspects in toxic neuropathies

Objectives Peripheral nerves are likely to be damaged by a wide array of toxins and medications causing different types of neuropathies mainly affecting distal nerves. Toxic neuropathies are often misdiagnosed due to the lack of available specific evidence. However, they can be suspected through clinical examination and electrodiagnostic features. We aimed to determine the pattern of clinical and electroneuromyographic disorder in toxic neuropathies. Methods A retrospective study was carried out on patients who had history of neurotoxin exposure and diagnosed with toxic neuropathy. All cases underwent detailed neurological examination and appropriate investigations needed to exclude other causes of neuropathy, as well as electrophysiological studies (EMG). Results Over the 21 patients we selected: 9 were alcoholic, 4 glue-sniffing addicts, 2 received medication with metronidazole and 3 had chemotherapy. Two had a professional exposure to N-Hexane and 1 did a suicide attempt with organophosphate. Neurological examination showed motor deficit predominating in distal limbs (61.9%), areflexia (85.7%) amyotrophy (19%) as well as proprioceptive ataxia (42.8%) and hypoesthesia (33.3%). Electrophysiological studies showed a symmetric sensorial dominant sensory-motor axonal type polyneuropathy (90%) with greater involvement of lower extremities (100%). Therefore, demyelinating features were detected in 40% with increased distal and F wave latencies (35%), decreased motor and sensory nerve conduction velocities (40%), motor conduction block (15%) and temporal dispersion (5%) defining the polyradiculoneuropathy electromyographic criteria (20%). Denervation signs were revealed in 30%. Most of patients (57.14%) regained their sensory and motor capacities within few months. Conclusion Peripheral neuropathies secondary to toxins are increasingly considered but can be difficult to definitively diagnose especially when it presents with a subacute severe form mimicking Guillain-Barre syndrome. Yet, detecting the syndromic presentation and a history of toxin exposure can facilitate prompt and accurate diagnosis.

Role of electromyography for the diagnosis of Parsonage-Turner syndrome

Objectives Parsonage-Turner syndrome (PTS) or neuralgic amyotrophy, is a rare idiopathic disorder characterized by acute onset of upper extremity pain, with subsequent development of muscle weakness and atrophy [1] , [2] . The pathogenesis is not fully understood, but immune-mediated process is thought to be a possible cause. We aimed to characterize the electro-clinical aspects observed in the PTS. Methods We included patients with neuralgic shoulder amyotrophy. All patients underwent physical and neurological examination. A complete biological assessment, lumbar puncture (PL), MRI of the cervical spine and electromyography (EMG) were performed in all cases. Results We report the cases of 3 patients, admitted to our neurological department for PTS. They respectively aged from 66, 20 and 72 years old. First case presented with a 2-month history of pain and 10-days history of weakness in her left wrist and hand. He reported the pain as sharp. Physical examination revealed marked atrophy and absence of strength in the shoulder muscles. Second case, came for subacute hands weakness. Neurological examination found asymmetric atrophy of the muscles of both shoulders. Third case, had sudden involuntary and brief rhythmic contraction involving upper and lower limbs mimicking myoclonus. Neurological examination noticed right shoulder atrophy and proximal muscle weakness. Biological assessment, study of cerebrospinal fluid and MRI of cervical spine were normal in all patients and excluded other causes of brachial neuritis. EMG typically consistent with PTS. It showed severe left total brachial plexopathy with acute axonal involvement and abundant acute denervation potentials, in the first case and a bilateral brachial plexopthy with severe axonal involvement in the other cases. All patients underwent high corticosteroid therapy with relative recovery. Conclusion PTS is a clinical diagnosis, however clinical presentation can sometimes lead to misdiagnosis. In such situation, the use of EMG would be a key exam to make the diagnosis of PTS.

Electric diagnosis of carpal tunnel syndrome: About 93 cases

Objectives Carpal tunnel syndrome (CTS) is a common clinical condition caused by entrapment of the median nerve (MN) at the flexor retinaculum of the wrist. A nerve conduction study (NCS) is one of the most sensitive and specific tools for diagnosing CTS. The purpose of our study is to determine epidemiological, clinical and various electrophysiological profiles of patients with CTS. Methods We collected the results of NCS of patients who had been addressed to the unit of electrophysiology in our department, for a period of one year, for clinically suspected CTS and we reviewed medical records of these patients. Results The sex ratio was 0.15. All patients presented acroparesthesia. All patients were right handed. CTS was bilateral and symmetrical in 15.5% of cases, bilateral and asymmetric in 16.3%, MN injury was present only in one side in 30.9%. NCS was normal for the rest. Axonal loss was noted in 38.8%. The correlation study showed that the presence of diabetes was associated with CTS severity and axonal loss. The severity of CTS and axonal loss were not correlated with duration of clinical progression and age of patients. Conclusion There was no correlation between clinical severity and electrophysiological profile witch can probably explain the discordance between preoperative electrophysiology scores of severity and postoperative recovery.

Guillain Barre Syndrome Associated with Brucellosis: A Case Report and Review of the Literature

Introduction: Guillain-Barre syndrome (GBS) ranks as the most frequent cause of acute flaccid paralysis in the world. It is an autoimmune polyradiculoneuropathy, usually preceded by an acute infection. Rarely, brucellosis may induce a GBS.Objective: To evaluate the clinical and microbiological diagnostic properties of Brucella-induced GBS.Case Report: A 54-year-old woman, with no past medical history, was followed in infectious disease department. She was diagnosed with brucellosis. She had received antibiotic therapy (Rifampicin 600 mg/day and Doxycycline 200 mg/day). After 4 days of treatment, she was referred to our department because of rapidly progressive, ascending, symmetric weakness and bilateral paralysis of muscles of the face. On admission, she was alert. The deep tendon reflexes (DTRs) were absent in all extremities. Muscle strength was 3/5 in the upper extremities and 2/5 in the lower extremities. Proprioception in the lower extremities was impaired, but she did not have any sensory problems. Our patient also presented a facial diplegia. Physical examination was normal, except for splenomegaly. A lumbar puncture showed an albumin-cytologic dissociation in the CSF. Nerve-conduction studies were suggestive of demyelinating polyradiculoneuropathy. Coombs Wright titration was 1/160..Discussion: With a diagnosis GBS preceded by brucellosis, our patient was given an antibiotic therapy (Rifampicin 600 mg/day and Doxycycline 200 mg/day). During hospitalization, she had four plasma exchange sessions and a motor rehabilitation. In a follow-up after 4 weeks, our patient presented a partial recovery, and she was able to walk without support.Conclusion: This case demonstrates that brucellosis can present with a rare neurologic manifestation including GBS. Molecular mimicry seems to be responsible for this complication, through the synthesis of autoantibodies against myelin gangliosides. Thus, brucellosis should be ruled out in all patients who develop acute flaccid paralysis, especially in those who live in endemic areas.