Syringomyelia Tubular cavitation of spinal cord, usually cervical, then other segments; more frequent in men; sometimes familial. Onset age 20 to 40. Slow progression; more rapid if brainstem affected (syringobulbia)….

Hereditary and Acquired Motor Neuron Diseases Definitions Diseases characterized by progressive degeneration and loss of motor neurons in spinal cord, motor nuclei of brainstem, motor cortex, or all three sites….

Hereditary and Acquired Spastic Paraplegia Hereditary Spastic Paraplegia Multiple inherited syndromes with prominent progressive leg weakness and spasticity. Complicated forms accompanied by additional clinical findings (Table 118.1). Genetics Genetically heterogeneous….

Tardive Dyskinesia and Other Neuroleptic-Induced Syndromes Adverse effects of drugs blocking D2 dopamine receptors (Table 117.1), especially antipsychotic agents (phenothiazines, butyrophenones); metoclopramide, flunarizine, cinnarizine. Likelihood of these adverse effects for…

Progressive Supranuclear Palsy Symptoms and Signs Usually present: pseudobulbar palsy (emotional lability), supranuclear ocular palsy (vertical gaze), square-wave jerks, extrapyramidal rigidity, gait ataxia, loss of postural reflexes, dementia. Early: falls….

Parkinsonism Characterized by any combination of: tremor at rest, rigidity, bradykinesia (slowing of movements), hypokinesia (less frequent spontaneous movements), flexed posture, loss of postural reflexes, “freezing” (motor block). Categories: idiopathic,…

Essential Tremor Chronic, progressive neurologic condition; 4 to 12 Hz tremor may involve arms, neck, voice, chin. Tremor primarily with movement (kinetic) or sustained posture. Rarely present at rest, in…

Dystonia Sustained muscle contractions, frequently cause twisting repetitive movements or abnormal postures (see also Chapter 9). Also called “torsion dystonia.” Movements sometimes rhythmic (dystonic tremor). EMG in dystonia: simultaneous contraction…

Gilles De La Tourette Syndrome Most common cause of tics. Diagnostic criteria: multiple motor and phonic tics; change in character over time; onset before age 21 years; symptoms wax and…

Myoclonus Brief, “lightning-like” muscle jerks with typically brief EMG bursts (20 to 100 msec). “Negative” myoclonus: sudden, brief lapse of contraction (e.g., asterixis). Prevalence 8.6/100,000. Differential diagnosis: no other movement…