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.

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.

SUMMARY 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.Stroke Case Reports Patient 1An 88-year-old man had a three week history of intermittent episodes of right-sided weakness and speaking difficulty. The episodes, lasting only seconds, involved weakness of the right arm and hand, buckling of the right knee, and speaking difficulty. The usual patterns were right arm and leg, sometimes only the right arm, and rarely the right leg alone. Thick and hesitant speech was associated with some of these events. Involuntary trembling movements of the right arm and hand frequently accompanied the right arm weakness, and prompted a trial of phenytoin treatment on the suspicion these were focal seizures. The phenytoin was discontinued, however, when he became toxic. There was no history of transient monocular blindness. A CT scan and EEG were both normal.On admission evaluation, blood pressure was 200/90. The general physical examination was negative except for loud bilateral carotid bifurcation bruits. The neurologic examination was remarkable only for an occasional hesitancy and stammering quality in his speech, and mild right lower face weakness. Strength in arms and legs was normal.During hospitalization four episodes of right arm and hand shaking were observed when the patient was either supine or sitting, all occurring during intravenous heparin anticoagulation. The movements were wavering, shaking excursions of the arm and hand which lasted 4-5 seconds. During one episode, there were 10 second wavering movements of the right hand, at which time he had difficulty holding a fork. He could talk during the shaking, although speech was more hesitant and several verbal paraphasic errors occurred. His right arm could be lifted on its own power to the horizontal during the shaking, but a lateral drift and wavering-shaking movements of a nonrhythmic nature were observed. Cerebral angiography demonstrated severe stenosis of the left internal carotid artery. Technical difficulties prevented further angiographic studies. Repeat CT scan...

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.

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.