Seyda Besen

Acute rhabdomyolysis associated with levetiracetam therapy in a child

Levetiracetam (LEV) is a new antiepileptic drug (AED) that is effective in adults and children with partial-onset seizures or idiopathic or symptomatic generalized seizures. LEV does not bind to plasma proteins, and is eliminated by the kidneys. It has been argued that LEV can act on the N-type Ca channel and can reverse the gammaaminobutyric acid (GABA) and glycine-gated currents [1]. Side effects include fatigue, somnolence, infection, headache, behavioral changes and skin rashes [2]. Herein, we present the first case of rhabdomyolysis in a child treated with LEV. A 13-year-old girl presented with a history of myalgia (mainly in her lower extremities) since last 2–3 days to our hospital. She was born of an uneventful full-term pregnancy. Developmental milestones were normal. On medical history, she had partial onset of secondarily generalized seizures during sleep since 2 months. The seizure activity consisted of twitching of the right face, tonic deviation of the mouth involving the lips, tongue, and pharyngeal and laryngeal muscles, resulting in speech arrest and drooling and progressing into bilateral tonic stiffening of the arms and legs. The EEG revealed left centrotemporal spikes and was subsequently diagnosed with benign epilepsy with centrotemporal spikes. The patient was administered LEV monotherapy. LEV had started 10 mg/kg/day, but her brief partial seizures continued. The dose was increased to 20 mg/kg/day (500 mg/day), and her seizures were ceased. One week after starting LEV therapy, she experienced mild myalgia. On her examination, the vital signs, including the blood pressure were normal. Her height and weight were in normal limit for her age. The physical and neurological examinations were normal. There was no evidence of trauma, exercise or infection. On laboratory examination, blood urea was 29 mg/dL (normal range 17–43), creatinine 1.0 mg/dL (normal range 0.6–1.2), myoglobin 78 ng/mL (normal range 14.3–65.8), and creatinine phospho kinase (CPK) was 986 U/L (normal range 27–168). Plasma carnitine (42 lmol/L; reference range 19–59 lmol/L) and free carnitine levels (35 lmol/L; reference range 12–46 lmol/L) were normal as acylcarnitine profile. Brain MRI showed no abnormal findings. Rhabdomyolysis was diagnosed with clinical and biochemical findings. We thought LEV may be the cause of her complaints and ceased LEV therapy. Hydration therapy were started to avoid potential complications. After stopping LEV, serum CPK and myoglobin levels gradually decreased and returned into normal values. Muscle biopsy was not performed. Several studies have reported that various AEDs may induce rhabdomyolysis [2, 3]. In the literature, few adult patients have been reported, but there have been no reports in children [4, 5]. Akiyama et al. [4] reported a 29-year-old woman with epilepsy in whom rhabdomyolysis was induced by LEV. The other case, a 19-year-old man, was described by Isaacson et al. [5]. Rhabdomyolysis is the destruction of skeletal muscle and release into the bloodstream of various cellular components, including CPK and myoglobin. The main causes of rhabdomyolysis may be direct muscle damage, intense physical exercise, certain drugs (some of them commonly used, such as statins), toxins, infections, and genetic & Faruk Incecik fincecik@cu.edu.tr

Limbic encephalitis with antibodies to glutamic acid decarboxylase presenting with brainstem symptoms.

Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. LE associated with glutamic acid decarboxylase antibodies (anti-GAD) is rare in children. Here, we characterized the clinical and laboratory features of a patient presenting with brainstem involvement with non-paraneoplastic LE associated with anti-GAD antibodies. In our patient, after plasma exchange, we determined a dramatic improvement of the neurological deficits.

Autoimmune encephalitis associated with glutamic acid decarboxylase antibodies: a case series

Antibodies against glutamic acid decarboxylase (GAD) are associated with various neurologic conditions described in patients including stiff person syndrome, cerebellar ataxia, refractory epilepsy, limbic and extralimbic encephalitis. GAD antibodies-related limbic encephalitis cases are well described; reports of extralimbic involvement are limited. We describe four cases of GAD antibody-related autoimmune encephalitis. Three of them had extralimbic involvement and only one had limbic encephalitis.

Finger drop sign in a child with acute motor and sensory axonal neuropathy form of Guillain-Barré syndrome.

Guillain–Barré syndrome (GBS) is an acquired acute autoimmune polyradiculoneuropathy. GBS has three subtypes defined by electrophysiological and pathogenetic findings, including acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN) [1]. We would like to report a patient with AMAN which showed a characteristic pattern of predominant finger extensor weakness. A 14-year-old boy was admitted to our hospital with numbness and weakness of his legs and arms lasting for 2 days. He reported upper respiratory system infection which onset 10 days prior to admission. There was no evidence of gastrointestinal system infection. On examination, he was conscious. Cranial nerves were intact. His muscle strength of both legs and arms was graded as 4/5 especially in distal muscles. He also had finger extensor weakness in hands (Fig. 1). Deep tendon reflexes were moderately brisk in four extremities and plantar responses were bilaterally in flexion. Sensory system examination showed hyperesthesia in the distal parts of the extremities. He remained ambulatory, with no evidence of bulbar and respiratory muscle weakness. Nerve conduction study was suggestive of AMSAN form of GBS (Table 1). He was treated with intravenous immunoglobulin (IVIg) (400 mg/kg/day) for 5 days and he recovered completely. The antibodies against Campylobacter jejuni which might cross react with gangliosides were negative in the serum and in the cerebrospinal fluid. In the literature, there is a report about weakness of finger extensors which has a certain distinguishing feature in AMAN form of GBS [2]. This pattern is called as ‘finger drop sign’ and consists of finger extensor weakness (i.e., at the metacarpophalangeal and interphalangeal joints) in the presence of relatively normal power in finger flexion, wrist flexion, and wrist extension. George et al. [2] reported a study including 12 cases of AMAN form of GBS. They observed that all AMAN patients showed a characteristic pattern of finger drop sign. It was the presenting symptom in four of the patients. They suggested that finger drop sign seemed to be relatively specific for the AMAN form of GBS. In the other case report, Dubey et al. [3] described a 9-year-old boy presented with finger drop sign with AMAN form of GBS. We observed finger drop sign in our patient with AMSAN form of GBS. He had no history of diarrhea and was also negative for antiganglioside antibodies against C. jejuni in the blood and in the cerebrospinal fluid. Finger drop sign was also reported among adults with lead neuropathy. Other differential diagnoses for finger drop sign include motor neuron disease (amyotrophic lateral sclerosis form), posterior interosseous nerve palsy, myasthenia gravis, syringomyelia and distal myopathies [4]. Absence of fasciculations and fluctuation of symptoms along with improvement with IVIg, make these diagnoses highly unlikely in the current case. In conclusion, we think that finger drop sign may be distinguishing features in axonal forms of GBS. & Faruk Incecik fincecik@cu.edu.tr

Guillain–Barré syndrome with hyperreflexia and bilateral papillitis in a child

Guillain–Barré syndrome (GBS) is an acute inflammatory polyneuropathy characterized by rapidly progressive symmetric weakness, and areflexia. Areflexia is necessary for the diagnosis of GBS. However, recently there have been studies of hyperreflexia with axonal neuropathy form of GBS. We report a 14-year-old boy with GBS, who presented with hyperreflexia and bilateral papillitis. To the best of our knowledge, this is the first pediatric patient presenting with papillitis and hyperreflexia with acute motor and sensory axonal neuropathy form of GBS.

Intravenous levetiracetam in critically ill children.

Background: To report the effectiveness and safety of intravenous (IV) levetiracetam (LEV) in the treatment of critically ill children with acute repetitive seizures and status epilepticus (SE) in a children′s hospital. Materials and Methods: We retrospectively analyzed data from children treated with IV LEV. Results: The mean age of the 108 children was 69.39 ± 46.14 months (1-192 months). There were 58 (53.1%) males and 50 (46.8%) females. LEV load dose was 28.33 ± 4.60 mg/kg/dose (10-40 mg/kg). Out of these 108 patients, LEV terminated seizures in 79 (73.1%). No serious adverse effects were observed but agitation and aggression were developed in two patients, and mild erythematous rash and urticaria developed in one patient. Conclusion: Antiepileptic treatment of critically ill children with IV LEV seems to be effective and safe. Further study is needed to elucidate the role of IV LEV in critically ill children.

P125 – 2716: A presentation of Lyme disease: Pseudotumor cerebri

Objective Lyme disease is caused by a tick-transmitted spirochete, Borrelia burgdorfer. It can present with both central and peripheral nervous system manifestations, including aseptic meningitis, meningoencephalitis, pseudotumor cerebri, Bells palsy and other cranial neuropathies, radiculoneuritis, and myelitis. Methods We reported a 8 year-old girl with pseudotumor cerebri secondary to Lyme disease. Results We treated ceftriaxone per day for the next 4 weeks, and acetozolamide. After teratment, her neurologic examination was normal. Conclusion Pseudotumor cerebri is a rare B. Burgdorferie related neurologic complication in childhood. Lyme disease should be included in the differential diagnosis of any child who presents with pseudotumor cerebri.

P61 – 2715: Limbic encephalitis with antibodies to glutamic acid decarboxylase presenting with brainstem symptoms

Objective Limbic encephalitis (LE) is a neurological syndrome that may present in association with cancer, infection, or as an isolate clinical condition often accompanying autoimmune disorders. LE associated with glutamic acid decarboxylase antibodies (anti-GAD) is rare in children. Methods We present a 15-yearold-boy with brainstem symptoms associated with anti-GAD antibodies. Results Intravenous immunoglobulins was administered. After intravenous immunoglobulins treatment, neurological condition did not improve. Then, we used plasmapheresis. After this treatment, we determined a dramatic improvement of his neurological symptoms. Conclusion The recognition of encephalitis associated with anti-GAD is important because of the potential response to the treatment.

P124 – 2714: Pseudotumor cerebri in a child with familial Mediterranean fever

Objective Familial Mediterranean fever (FMF) is an autosomal recessive polysystemic disease characterized by attacks of relapsing and self-limiting fever, peritonitis, pleuritis and arthritis. Neurologic involvement is rare but serious in FMF. Headache, seizures, demyelinating lesions, stroke, posterior reversible leukoencephalopathy syndrome, aseptic meningitis, convulsions, and cranial neuropathy have been reported in previously literature. Methods We reported a 10 year-old boy with pseudotumor cerebri and FMF. Results We treated acetozolamide. After treatment, we described a dramatic improvement of his neurological symptoms. Conclusion Pseudotumor cerebri may be a manifestation of FMF in children. Early diagnosis is important for preserving visual functions.