Tremor

a rhythmic involuntary oscillation of a body part , produced by either alternating or synchronous contractions of reciprocally innervated antagonistic muscles.
Tremor
1 - Resting tremors Parkinson's disease Essential tremor11-Postural tremors exaggerated physiological tremor Endocrine hypoglycemia – hyperthyroidism -heochromocytoma Drugs and toxins adrcnocorticosteroids, beta agonists, dopamine agonists, amphetamines, lithium, tricyclic antidepressants, narcoleptics, theophylline, caffeine, valproic acid, alcohol withdrawal, mercury ,lead, arsenic, 111--Intention tremors Cerebellar tremor Types

fine tremor of the outstretched limbs usually not noticeable except with electrophysiological recording
accentuated by fatigue, anxiety, fear, excitement, stimulant use, and medical conditions such as hyperthyroidism
Essential Tremor
Physiological Tremor
higher in men than in women and in whites Bilateral, largely symmetrical postural or kinetic tremor in hands/forearms that is visible and persistent ,Additional or isolated head tremor may occur ; two thirds of patients give a positive family history of tremor
B-adrenergic blockers and primidone and anticholinergic medications.
combination therapy may be more effective Stereo tactic thalamotomy Thalamic DBS


Neurological parkinsonism, postural and kinetic. tremor, ataxia, titubation chorea, seizures, dysarthria, or dystonia.
an autosomal recessive disorder of copper metabolism related to mutations in the ATP7B gene on chromosome 13
liver disease,ranging from cirrhosis to fulminant liver failure
Ophthalmologic :including slit-lamp examination essentially always demonstrates copper deposition in Descemet's membrane (Kayser-Fleischer rings] KF ring
Psychiatric ; behavioral psychosis


symptoms usually appear in the 2nd - 3rd decade. The first signs are hepatic in about 40% of cases, neurological in about 35%


1. WD should be considered in any individual between the ages of 3 and 55 years with liver abnormalities of uncertain cause. 2. WD must be excluded in any patient young with neurological or neuropsychiatric disorder. 3. In a patient in whom WD is suspected, Kayser- Fleischer rings should be sought by slit-lamp examination by a skilled examiner. The absence of Kayser- Fleischer rings does not exclude the diagnosis of WD, even in patients with predominantly neurological disease


Treatment ZINK – PENICILLAMINE: TRIENTINE Investigation Low serum ceruloplasmin, elevated 24-hour copper excretion the presence of Kayser-Fleischer ring Penicillamine challenge studies elevated hepatic copper
screeining Low serum ceruloplasmin, the presence of Kayser-Fleischer ring


means "a dance it is irregular, unpredictable , semi-purposive brief, jerky movements that flit randomly from one part of the body to another
CHOREA
Hereditary choreas Huntington's disease Benign hereditary chorea Neuroacanthocytosis
Neurometabolic disorders: Wilson's disease,, lysosomal storage disorders, amino acid disorders hyperthyoidism
Drugs: neuroleptics (tardive dyskinesia), anti-parkinsonian drugs, amphetamines, cocaine, tricyclics, oral contraceptives Toxins: alcohol intoxication and withdrawal, anoxia, carbon monoxide, manganese, mercury, thallium,
Infectious and post infectious Sydenham's chorea Encephalitis lethargica
Pregnancy (chorea gravidarum]
Systemic lupus erythematosus Stroke Henoeh-Schonlein purpura Tumor

eyebrow lifting or depression, lid winking, lip pouting or pursing, cheek puffing, lateral or forward jaw movements, tongue rolling or protruding, head jerking in any plane (a common pattern is a sudden, backward jerk followed by a rotatory sweep forward), shoulder shrugging, trunk jerking or arching, pelvic rocking, and flitting movements of the fingers, wrists, toes, and ankles
the gait is irregular and lurching
Tone usually is normal to low
motor impersistence (e.g., difficulty maintaining eyelid closure, tongue protrusion, or constant hand grip
"Milkmaid grip
Huntington's Disease
Completely penetrant autosomal dominant disease characterized by progressive movement disorder chorea psychiatric and cognitive decline euphoria agitation hallucination delusion terminal state of dementia and immobility dementia
10 per 100,000
begins between the ages of 30 and 55



Clinical features Personality and behavior changes Dementia more sever in early onset Chorea Bedridden
Atrophy of
Cerebral cortex Corpus striatum
Dementia –psychological chorea Blunted drive aggressiveness Coarsening of personality
Death – 15 years 5% presents with akinetic rigid Parkinson syndrome ;commonest in children [westphal variant] Epilepsy may be a feature Investigation MRI– CT scan caudate atrophy DNA analysis

Sydenham's Chorea :

appears months after the index infection ;affects girls more than boys, between the ages of 5 and 15 years ; appears 3 months after onset of Rheumatic fever


Ataxia imbalance and incoordination involving either gait and limbs it is either due to a disorder involving the cerebellum or its connections or due to proprioceptive disorder Types
truncal ataxia gait ataxia appendicular ataxia

sensory ataxia Cerebeller ataxia
Neurological Signs
Stance and gait : tandem gait Romberg test
Ataxic gait is characterized by a widened base and an irregular staggering appearance

2. Limb incoordination

finger-to-nose test
heel-to-shin maneuver
Dysmetria under-reached (hypometria) or over-reached (hypermetria
Dysdiadochokinesis: This term refers to irregularity of the rhythm and amplitude of rapid alternating Movements
Decomposition of movements;
Rebound phenomena
Romberg test + Impaired position and vibration sense and the deep tendon reflexes
Dys synergia

Friedreich '$ Ataxia spinocerebeller disease

age at onset of younger than 25 years, typically early in adolescence
1--Onset is with increasing gait difficulties and gait ataxia, loss of proprioceptive sense in the lower limbs, and absence of deep tendon reflexes, 2- Skeletal deformity pes cavus
3--upper motor neuron findings such as extensor plantar responses and 4- Optic atrophy
5-Rarely, patients may present with cardiac disease or .: spinal deformity


profound proprioceptive loss, areflexia, weakness of lower limb muscles, dystonia, flexor spasms, and increasing dysarthria and dysphagia Optic atrophy and hearing loss may occur in many patients Systemic abnormalities abnormal electrocardiogram ecordings, hypertrophic cardiomyopathy in about 50% of the patients, and diabetes in 10%. Skeletal abnormalities such as spinal deformities and foot deformities are common

The mean age at death --late in the fourth decade---Cause of death is usually cardiac

MRI scans of the brain reveal no abnormalities in the cerebellum; rather, the upper cervical cord shows atrophy
Nerve conduction studies show early absence or reduction of sensory nerve potentials in a diffuse fashion
A tax ia-Telangiectasia the disease has its onset in the first decade. Children develop progressive ataxia associated with hypotonia, areflexia, peripheral neuropathy, and choreoarhetosis
Telangiectasias develop over the conjunctivae ,ear lobes, and other areas during the second half of the first decade.
Many patients have decreased immunoglobulin A (IgA) levels; decreased munoglobulin E and immunoglobulin M levels, lymphocytopenia
The Fried Reich's Ataxia Mutation===the disease is recessively inherited on chromosome 9


Ataxia with Isolated Vitamin E Deficiency childhood-onset recessive ataxia associated with isolated vitamin E deficiency
causing ataxia, areflexia, proprioceptive loss, and dysarthria
lower incidence of cardiomyopathy and more frequent head titubation, compared with patients with FA Vitamin F levels should be obtained in all persons with sporadic ataxia of childhood or young adult onset. Patients withAVED have typically less than 1.8 mg/L of vitamin E in serum. Treatment with large doses of vitamin E