George D. Baquis

Dissection of the intracranial vertebral artery

We describe four patients and review prior reports to clarify the clinical, radiographic, and pathologic findings of intracranial vertebral artery (VA) dissection. A 43-year-old man and a 33-year-old woman had chronic bilateral VA dissecting aneurysms. The man had multiple episodes of subarachnoid hemorrhage (SAH) and necropsy showed multiple dissections and defects in the internal elastica. The woman had many brainstem TIAs and strokes during 3 years. Two other patients had SAH and unilateral dissections. Intracranial VA dissection causes four overlapping syndromes: (1) brainstem infarcts are usually due to subintimal dissection extending into the basilar artery, affect younger patients, and often are single fatal events; (2) SAH is due to subadventitial or transmural dissection; (3) aneurysms cause mass effect on the brainstem and lower cranial nerves; and (4) chronic dissections due to connective tissue defects cause extensive bilateral aneurysms and repeated TIAs, small strokes, and SAH.

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis

Objective: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. Methods: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. Results: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). Conclusions: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.

Limb shaking--a carotid TIA.

Eight patients are described with an unusual form of carotid transient ischemic attack, limb shaking. The basic features included a brief, involuntary, coarse, irregular, wavering movement or tremble involving arm-hand alone, or arm-hand and leg together. In 2 patients limb shaking was the initial manifestation of carotid occlusive disease, and all but one patient had other typical carotid transient ischemic attacks. Major atheromatous carotid occlusive disease was present in all patients on the side opposite the limb movements. Four patients had bilateral carotid occlusive disease. Cerebral ischemia from a carotid territory low-perfusion state may be the pathogenesis of these limb movements, an idea supported by the apparent benefit of surgical revascularization in abolishing or reducing the limb shaking in 6 patients. There was no clinical or EEG evidence to document an epileptiform etiology. Recognition of this uncommon form of carotid transient ischemic attack may be important in the early diagnosis and treatment of carotid occlusive disease.

Dental chair intracerebral hemorrhage

A 52-year-old woman had a fatal intracerebral hemorrhage after dental manipulation. Normotensive in the past, the initial blood pressure was high but rapidly returned to normal. Necropsy showed no vascular malformation or evidence of hypertensive vascular disease. Clinical and experimental data show that stimulation of trigeminal fibers can cause important changes in blood pressure and pulse.

Congenital Guillain-Barré Syndrome

5. Connor CL, Cushing H: Diffuse tumors of the leptomeninges: Two cases in which process was revealed only by microscope. Arch Pathol 1927;3:374-392. 6. Whelan HT, Mastri AR: Primary diffuse meningeal gliomatosis in children. Ann Neurol 1984;16:391. 7. Canady A, Zakalik K: Brain tumors presenting as meningitis. Presented at the First International Pediatric Neuro-Oncology Conference, June 1989.

Ventral polyradiculopathy with pediatric acute lymphocytic leukemia.

A 3‐year‐old girl with acute lymphocytic leukemia (ALL) in remission developed lower extremity paraparesis and areflexia 15 days after receiving intrathecal methotrexate, cytarabine, and hydrocortisone. Cerebrospinal fluid protein was 107 mg/dl. Compound muscle action potential amplitudes were reduced, F waves were absent, and sensory conduction studies were normal. Needle electromyography (EMG) revealed reduced motor unit potential recruitment. Magnetic resonance imaging (MRI) showed lumbosacral ventral root enhancement. She was treated with intravenous immunoglobulin and slowly recovered. Nerve conduction and EMG abnormalities correlated with MRI root enhancement, facilitated early diagnosis, and distinguished this from a myelopathy or distal polyneuropathy. These findings could represent selective ventral nerve root vulnerability to intrathecal chemotherapy. A selective autoimmune process cannot be excluded.

Acute brachial diplegia due to Lyme disease.

ObjectiveTo describe acute brachial diplegia as the initial manifestation of Lyme disease. BackgroundBilateral, predominantly motor, cervical radiculoplexus neuropathy, the “dangling arm syndrome,” has not been reported as a complication of acute Lyme infection. MethodsRetrospective series of 5 patients from 2 tertiary neuromuscular centers. ResultsThere were 4 men and 1 woman with an average age of 69 years. One recalled a tick bite, and preceding constitutional symptoms included headache (2) and fever, arthralgias, and fatigue in 1 patient each. Proximal arm weakness and acute pain developed within 3 weeks from onset; pain was bilateral in 3 patients and unilateral in 2 patients, and was described as severe throbbing. Arm weakness was bilateral at onset in 3 patients, and right sided in 2 patients followed by spread to the left arm within days. All the patients had weakness in the deltoid and biceps that was 3/5 or less (Medical Research Council scale), with variable weakness of the triceps and wrist extensors; 1 patient had a flail right arm and moderate (4/5) weakness of the proximal left arm muscles. Light touch was normal in the regions of weakness, and 1 patient had mildly reduced pin sensation over the forearm. Serum IgM Lyme titers were elevated in all the patients and were detected in the cerebrospinal fluid in 4 tested patients. The cerebrospinal fluid protein ranged between 135 and 176 mg/dL with lymphocytic pleocytosis (range, 42 to 270 cells). Electrodiagnostic studies showed normal median and ulnar motor potentials with asymmetrically reduced sensory amplitudes in the median (4), ulnar (3), and radial, and lateral antebrachial cutaneous potentials in 1 patient each. Two patients had acute denervation in the cervical or proximal arm muscles. There was full recovery after antibiotic therapy in 4 patients and considerable improvement in 1 patient after 2 months. ConclusionAcute brachial diplegia is a rare manifestation of acute Lyme infection and responds promptly to antibiotic therapy.

Pressure-related torticollis: An unusual manifestation of pseudotumor cerebri

A 9-year-old boy with pseudotumor cerebri who presented with neck pain and an accompanying torticollis is described. The patient exhibited bony abnormalities of the upper cervical spine and facial asymmetry that suggested a congenital torticollis. Reduction of the increased cerebrospinal fluid pressure by lumbar puncture resulted in a prompt and dramatic resolution of the cervical symptoms and signs. Increased cerebrospinal fluid pressure should be added to the list of those disorders that may give rise to reversible torticollis.

Turning Electromyography Reports Upside Down: A Pilot Study Surveying Referring Providers

Providers are expressing a desire for more efficient ways to retrieve relevant clinical data from the Electronic Health Record. In an effort to improve our Electromyography and Nerve Conduction Study reports, we surveyed referring providers on the effects of having the IMPRESSION at the start of the report. Our survey respondents felt that using this format for an Electromyography and Nerve Conduction Study report significantly improved the quality of the report while saving them time and/or mouse clicks when interpreting the report. Electro diagnosticians might consider using this format for their Electromyography and Nerve Conduction Study reports to improve referring provider satisfaction.