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Results for “REFLEX, BABINSKI”

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At least 145 records · Page 8Linked to original sources
PubMed2003

Phenotypic features and genetic findings in sacsin-related autosomal recessive ataxia in Tunisia.

BACKGROUND: Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a clinically homogenous disorder reported in Quebec caused by mutations in the SACS gene (chromosome 13q12). Recently, we identified a Tunisian kindred demonstrating linkage to the ARSACS locus. OBJECTIVE: To report clinical, neurophysiological, and nerve biopsy findings in patients with autosomal recessive cerebellar ataxia related to the SACS gene in Tunisia. PATIENTS AND METHODS: Genetic linkage analysis of patients with early-onset autosomal recessive cerebellar ataxia allowed the identification of 4 families from which 18 patients demonstrated linkage to the ARSACS locus. The patients were evaluated according to the International Cooperative Ataxia Rating Scale. Peripheral nerve conduction, sensory evoked potentials, and nerve biopsy were performed in most patients. RESULTS: The mean age at onset was 4.5 years. The clinical phenotype was stereotyped and associated with a progressive cerebellar syndrome, a pyramidal syndrome with brisk knee reflexes, and Babinski sign and absent ankle reflexes. The course of the disease varied among patients. Sensory evoked potentials showed severe posterior column involvement. Peripheral nerve investigations demonstrated axonal and demyelinating neuropathy. Four mutations, 2 missense and 2 nonsense, were found. CONCLUSION: In Tunisia, autosomal recessive cerebellar ataxia related to the SACS gene demonstrated a homogenous phenotype and heterogeneous allelic mutations.

Adolescent↗
PubMed2002

The "bedsheet" Babinski.

BACKGROUND: Babinski's reflex (dorsiflexion of the hellus and fanning of the toes in response to plantar stimulation) is a classic sign in neurology. Although typically elicited by stroking the plantar surface of the foot with a firm object, other means of obtaining the response have been described. METHODS: We assessed the frequency of the Babinski response after plantar stimulation by exposing the feet by removal of the bedsheet or socks in 10 patients with neurologic disease and 10 control subjects. These results were compared with those obtained by traditional means of stimulation. RESULTS: We found a high correlation between the two responses (sensitivity of 80% and specificity of 90%). CONCLUSION: Although this study is small and requires a larger study to verify its accuracy, it appears that simple observation of the toe response at the time that the feet are exposed frequently provides evidence of pyramidal tract dysfunction.

Adult↗
PubMed1996

[A case of acute disseminated encephalomyelitis (ADEM) associated with demyelinating peripheral neuropathy].

A 41-year-old man had common cold on April 10, 1991. Five days after this illness he developed fever, dysarthria, visual field impairment, gait disturbance and consciousness disturbance followed. On admission in another hospital, cerebrospinal fluid showed 341/mm3 cells (303 mononuclear cells, 33 polynuclear cells, 5 red cells), protein of 238 mg/dl, and sugar of 59 mg/dl. One month later, the neurological examinations revealed flaccid paraparesis, decreased deep tendon reflexes in the lower extremities, bilateral positive Babinski and Chaddock reflexes, positive Beevor sign, stocking type superficial sensory disturbance, diminished vibration sense in the lower extremities and neurogenic bladder. Eight months later, he improved to be able to walk by himself, but decreased Achilles tendon reflexes, bilateral positive Babinski and Chaddock reflexes and paresthesia over both feet were noted neurologically. Nerve conduction study revealed reduced conduction velocities at tibialis anterior nerves, sural nerves and no response on both deep peroneal nerves. A 1 micron thick epon section of a biopsied sural nerve with toluidine blue stain showed a decreased number of myelinated fibers (6394/mm2) with many thinly myelinated fibers and Renaut bodies. There was no edema nor cell infiltration. Electronmicroscopical findings of their ultrathin sections showed many collagen pockets, denervated Schwann cell clustering and a few onion bulb formations. Teased fiber preparations suggested segmental demyelination and remyelination in many fibers. This case could be regarded as a case of ADEM associated with demyelinating peripheral neuropathy, and the possibility of the simultaneous demyelinating process in the central and peripheral nervous system was discussed.

Adult↗
PubMed1976

Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia.

The 50 patients in this survey were classified by a panel of neurologists into 4 clinical sub-groups: Group Ia ("typical" Friedreich's ataxia, complete picture), Group Ib ("typical" Friedreich's ataxia, incomplete picture), Group IIa ("atypical" Frriedreich's ataxia, possible recessive Roussy-Levy syndrome), Group IIb (heterogeneous ataxias). The clinical symptoms and signs were analyzed for each of these groups. A constellation of signs constantly present in Friedreich's ataxia and obligatory for diagnosis was described. Other important symptoms, such as the Babinski sign, kyphoscoliosis and pes cavus were found to be progressive, but not essential for the diagnosis at any given time. Finally, a host of other symptoms can only be called accessory. The progression of scoliosis was found to be an important tool in the differential diagnosis of ataxias. Our study also indicates, in contrast to the opinion of some authors, that absent deep tendon reflexes in the lower limbs and early dysarthria are essential in "typical" Friedreich's ataxia.

Adolescent↗
PubMed1993

Babinski's sign.

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Humans↗
PubMed1976

The significance of the Babinski sign in the newborn-a reappraisal.

A study of the clinical significance and the stimulus methods used to elicit the Babinski sign in normal newborns has been carried out. Although the most frequent response to cutaneous nociceptive stimulation of the lateral sole in neonates was extension of the great toe, a small but significant number of flexor responses were obtained. Consequently, the Babinski sign has limited clinical usefulness as a reflex indicating the presence or absence of neurological disease in the newborn. In reviewing previous studies in the literature, it was apparent that the widely differing percentages of extensor plantars reported in newborns was related directly to differences in the technique of examination.

Humans↗