Isaac Syndrome with Intractable Neuropathic Pain Features: A Case Report.

Isaac syndrome (IS) is a peripheral nerve hyperexcitability state associated with voltage-gated potassium channel (VGKC) complex antibodies. Major manifestations are muscle twitching, stiffness, hypertrophy, and dysautonomic features such as hyperhidrosis [Ahmed and Simmons. Muscle Nerve. 2015;52(1):5-12]. Neuropathic pain is a rare manifestation. We describe a case of IS characterized by muscle twitching and intractable neuropathic pain. Diagnostic workup included elevated VGKC complex antibodies and EMG/NC that showed neuromyotonic discharges. Neuropathic pain was initially difficult to relieve even after using multiple medications, including opiates, benzodiazepines, anticonvulsants, and intravenous immunoglobulin (IVIg). Moderate pain control was eventually achieved with long-term use of carbamazepine and subcutaneous immunoglobulin (SCIg). Common manifestations of IS are muscle twitching, stiffness hypertrophy, and dysautonomia [Ahmed and Simmons. Muscle Nerve. 2015;52(1):5-12]. Sensory manifestations such as neuropathic pain are rare, but, as illustrated by our patient, can be the most distressing symptom. In our patient, not only was neuropathic pain disabling but it also showed the least response to IVIg. The use of 200 mg of long-acting carbamazepine twice daily with weekly SCIg demonstrated the best response. This case highlights an uncommon but potentially resistant symptom of IS.

Acute Lymphoblastic Leukemia Masquerading as Guillain-Barré Syndrome.

INTRODUCTION: Neurological manifestations of acute lymphoblastic leukemia (ALL) have been reported as cranial neuropathies or meningeal symptoms most common in children. However, ALL can rarely involve the nerve roots causing symmetrical polyradiculopathy which can present with rapid onset paralysis, mimicking Guillain-Barré Syndrome (GBS). The symmetrical polyradiculopathy can be the earliest manifestation of ALL occurring even before the hematological and systemic manifestations. CASE REPORT: We report a case of a healthy 29-year-old man who presented with subacute bilateral lower extremity weakness and numbness preceded by a respiratory infection. He was initially treated as a suspected (GBS) but cerebrospinal fluid (CSF) findings suggested an alternative diagnosis. His prior TB exposure created a diagnostic confusion. Lumbar spine magnetic resonance imaging revealed nerve root enhancements at L4-L5 and L5-S1 that are seen in GBS and TB arachnoidids. Brain magnetic resonance imaging demonstrated bilateral distention of the optic nerve sheath complexes with CSF suggestive of intracranial hypertension. CSF revealed elevated protein, nucleated cells 2145 leukocytes/mm 3 , numerous atypical lymphoid cells. He was later diagnosed with ALL associated symmetrical polyradiculopathy presenting with GBS-like symptoms. CONCLUSION: Symmetrical polyradiculopathy is a rare complication of ALL and can be confused with acute inflammatory demyelinating polyneuropathy. ALL associated polyradiculopathy in young individuals can be clinically indistinguishable from GBS. Our case highlights that when CSF findings are atypical for GBS, ALL should be considered on the differential diagnosis in patients presenting with GBS like symptoms.

Neurologic complications after surgery for obesity.

Bariatric surgical procedures are increasingly common. In this review, we characterize the neurologic complications of such procedures, including their mechanisms, frequency, and prognosis. Literature review yielded 50 case reports of 96 patients with neurologic symptoms after bariatric procedures. The most common presentations were peripheral neuropathy in 60 (62%) and encephalopathy in 30 (31%). Among the 60 patients with peripheral neuropathy, 40 (67%) had a polyneuropathy and 18 (30%) had mononeuropathies, which included 17 (94%) with meralgia paresthetica and 1 with foot drop. Neurologic emergencies including Wernicke's encephalopathy, rhabdomyolysis, and Guillain-Barré syndrome were also reported. In 18 surgical series reported between 1976 and 2004, 133 of 9996 patients (1.3%) were recognized to have neurologic complications (range: 0.08-16%). The only prospective study reported a neurologic complication rate of 4.6%, and a controlled retrospective study identified 16% of patients with peripheral neuropathy. There is evidence to suggest a role for inflammation or an immunologic mechanism in neuropathy after gastric bypass. Micronutrient deficiencies following gastric bypass were evaluated in 957 patients in 8 reports. A total of 236 (25%) had vitamin B(12) deficiency and 11 (1%) had thiamine deficiency. Routine monitoring of micronutrient levels and prompt recognition of neurological complications can reduce morbidity associated with these procedures.

Complete heart block with ventricular asystole during left vagus nerve stimulation for epilepsy.

Vagus nerve stimulation (VNS) is an important therapeutic option for individuals with refractory epilepsy who have failed multiple antiepileptic drugs (AEDs). The intricate relationship of the vagus nerve to cardiac function raises concern that vagal stimulation may affect cardiac rhythm and function. Previous pre- and postmarketing studies have not shown this to be a significant problem, with the incidence of bradyarrhythmias reported to be about 0.1%. We review three cases of ventricular asystole with complete heart block that occurred during intraoperative lead tests. The purpose of these case reports is to identify the specific type of cardiac abnormality associated with vagus nerve stimulation and to identify individuals at risk.