Lhermitte's sign. From observation to eponym.

The Lhermitte's sign was first described by Pierre Marie and Chatelin in 1917. Lhermitte reported on this symptom in 1920, and in 1924 he published the seminal article on the subject. In 1928, it was introduced to the American literature, and it was around that time that the symptom became well known. The historic development of this observation into an eponym is documented.

Anatomic-radiologic basis of Lhermitte's sign in multiple sclerosis.

OBJECTIVE: To test the presumption that Lhermitte's sign in multiple sclerosis is the result of a lesion in the cervical spinal cord. DESIGN: The radiologic files of 887 patients with multiple sclerosis were reviewed. A detailed questionnaire regarding Lhermitte's sign was sent to 75 patients who had undergone magnetic resonance imaging of the brain and cervical spinal cord. Of the 64 patients who responded, 55 patients who had complete magnetic resonance imaging files were studied. The cases of two illustrative patients are presented. RESULTS: A strong association between Lhermitte's sign and abnormalities of the cervical spinal cord seen on magnetic resonance imaging was noted. Most of the abnormalities were in the posterior part of the cervical spinal cord. CONCLUSIONS: The findings confirm the presumption that a lesion in the posterior columns of the cervical spinal cord is the cause of Lhermitte's sign in multiple sclerosis.

Reflex epilepsy and nonketotic hyperglycemia in the elderly: a specific neuroendocrine syndrome.

We present five elderly patients with focal reflex or posture-induced seizures and nonketotic hyperglycemia (NKH). Each patient exhibited interictal focal findings, such as hemiparesis or hemisensory or aphasic deficits. With control of the hyperglycemia, the seizures stopped, and the neurologic deficits resolved. The syndrome of focal reflex epilepsy and neurologic deficits in the elderly is transient and almost invariably related to NKH, thus representing a specific neuroendocrine syndrome.

Pyramidal tract deficits and polyneuropathy in hyperthyroidism, Combination clinically mimicking amyotrophic lateral sclerosis.

Generalized weakness, intermittent dysphagia, and a 40-pound weight loss developed in an elderly man over a six-month period. Examination revealed weakness, atrophy and fasciculations of extremity musculature, pseudobulbar speech, hyperactive upper extremity reflexes, and extensor toe signs without sensory loss. Results of electrodiagnostic studies were consistent with an axonal polyneuropathy. Endocrinologic results were compatible with hyperthyroidism. Radioiodine therapy resulted in resolution of clinical neurologic symptoms and signs within seven months. This case illustrates a previously undescribed concurrence of hyperthyroid associated polyneuropathy and pyramidal tract dysfunction that led to an initial clinical diagnosis of amyotrophic lateral sclerosis.

Retinal migraine, chorea, and retinal artery thrombosis in a patient with primary antiphospholipid antibody syndrome.

We report the case of a patient with the unusual combination of migraine, chorea, and retinal arterial thrombosis along with laboratory evidence of autoimmunity. In the absence of systemic lupus erythematosus, the clinical manifestations suggest the presence of the primary antiphospholipid antibody syndrome.

Sympathetic skin response in multiple sclerosis.

Somatosympathetic reflex was studied in 29 patients with definite multiple sclerosis (MS) by the non-invasive sympathetic skin response (SSR) method. Abnormal SSRs in 1 or more limbs were noted in 17 patients. Good correlation between the number of absent SSRs and the severity of the disability caused by MS was observed. Delayed or absent SSRs correlated with leg weakness, spinothalamic sensory deficits, and neurogenic bladder. No correlation was noted between signal abnormalities seen on magnetic resonance imaging studies of the hypothalamus or brain stem and absent or delayed SSRs. It is suggested that damage to the central sympathetic fibers in the spinal cord accounts for the absent SSR. The damaged sympathetic fibers are probably located in the lateral columns of the spinal cord.

Sympathetic skin response.

The sympathetic skin response (SSR) is the potential generated by sweat in response to different stimuli. This potential has a waveform that habituates with closely repeated stimuli and a latency of 1.3-1.5 s at the hand and 1.9-2.1 s at the foot. It has been used to study the peripheral sympathetic system in peripheral nerve diseases. The response is absent in many cases of peripheral neuropathy, often in the presence of autonomic symptoms. SSR has also been used to study a few diseases that may affect the central sympathetic system. This review outlines the history, physiopathogenesis, and analysis of SSR as well as general techniques used in its study. The reported association of SSR with peripheral and central nervous system diseases is also presented.

International regulations for automobile driving and epilepsy.

BACKGROUND: Many patients with epilepsy travel abroad and drive automobiles with the assumption that policies, rules, and regulations on epilepsy and driving are similar to those of their home countries. This paper investigates the driving restrictions and other pertinent information on this issue in foreign countries. METHODS: A questionnaire was sent to 231 neurologists (chosen from American neurological and epilepsy societies) from 84 countries and to 230 official (embassies and consulates) representatives of 134 countries asking for the local rules and regulations and their comments on driving and epilepsy. RESULTS: One hundred and sixty-six responses were received from 96 of 134 (72%) countries. One hundred and six neurologists (of 231 queried [46%]) responded. In 16 countries, persons with epilepsy are not permitted to drive. In the remaining countries, these patients must have a seizure-free period of 6 to 36 months. This period varies according to the type of seizure. In five countries, physicians must report the names of these patients to their local authorities. In many countries, the rules and regulations are being reevaluated and changed. CONCLUSIONS: Patients with epilepsy who plan to drive overseas are advised to contact local embassies and consulates, well before their trips (and keep records of the communications) to obtain the latest information on the rules and regulations governing the driving of automobiles in those countries.

Autonomic cardiovascular reflexes in progressive supranuclear palsy.

Assessment of possible autonomic nervous system dysfunction was performed by testing cardiovascular reflexes in nine patients with progressive supranuclear palsy (PSP). The patients were significantly different from 15 control age-matched subjects because of greater blood pressure drop on standing for 1 min, diminished rise of diastolic blood pressure during the sustained handgrip test, and lack of compensatory tachycardia measured by the 30:15 ratio on standing. The latter test abnormality was caused by a slow rise to erect posture. No differences were observed in the cold pressor test, Valsalva ratio, and heart rate response to deep breathing. The differences between patients and control subjects were usually small. Autonomic nervous system involvement in patients with PSP is minor and is caused by involvement of central brain stem sympathetic nuclei or efferent fibers.

Clinical implications of benign epileptiform transients of sleep.

One hundred electroencephalograms (EEGs) with benign epileptiform transients of sleep (BETS) were reviewed. The incidence of epileptic seizures, syncope, headaches, transient ischemic attacks (TIAs), dizziness and psychiatric complaints was determined. The incidence of these symptoms was similarly ascertained for another group of patients whose sleep EEGs had preceded (96 patients) or followed (96 patients) each of the BETS EEGs. These patients formed 2 separate internal control groups. No significant differences in the demographic composition of the 3 groups or in the incidence of symptoms or diagnoses were noted. The patients with BETS were also divided into 2 groups according to whether their EEGs were otherwise normal or abnormal. Each of these 2 groups was compared with their respective control group (pre- or post-BETS patients with normal or abnormal EEGs). No significant differences in the incidence of seizures were noted. Benign temporal epileptiform transients of sleep appear to be an occasional but clinically unimportant finding in sleep tracings.

Bilateral hypoglossal nerve injury after bilateral carotid endarterectomy.

A case of severe bilateral injury to the hypoglossal nerves after two-stage carotid endarterectomy is described. Injury to the hypoglossal nerve occurs in up to 20% of patients undergoing carotid endarterectomy and may result in mild or unnoticed deficits. These injuries must be carefully searched for in patients who will undergo a similar procedure on the opposite side since a bilateral deficit of the hypoglossal nerve is poorly tolerated, causing potentially serious impairment of speech and risk of aspiration.