Westphal's Symptoms

WESTPHAL'S SYMPTOMS, SYNDROME (K.K. Westphal). Westphal's symptoms: 1. Knee reflex - absence of the knee (patellar) reflex. This symptom has enormous diagnostic significance. One can speak of the absence of the knee reflex when it cannot be elicited despite repeated examinations and the use of different auxiliary methods (diversion of attention according to Jendrassik, according to Wahlbaum). Mechanical causes must also be excluded (disease of the knee joint, dislocation of the tendon, significant fat deposition, edema, etc.). Absence of the knee reflex is observed in diseases of the central nervous system (tabes dorsalis, progressive paralysis, diseases of the anterior horns of the spinal cord), in diseases of the peripheral nervous system (radiculitis, neuritis), in myopathies. The reflex may also be absent in certain other conditions; for example, during deep anesthesia, with increased intracranial pressure, and generally during deep loss of consciousness. Extreme elevation of temperature and severe fatigue due to excessive physical exertion can also have this effect; absence of the reflex is also observed when an Esmarch bandage is applied. In very rare cases, the knee reflex may be absent in healthy people from birth. Pathological weakening of the knee reflexes can be spoken of when they are not clearly elicited even with the use of auxiliary methods, or if the contraction extends only to individual parts of the quadriceps muscle of the thigh (for example, m. vastus internus). 2. Paradoxical muscle phenomenon consists of tonic contraction of the m. tibialis anticus and dorsal flexors during abrupt passive dorsal flexion of the foot, as a result of which the latter remains in a state of dorsal flexion for some time. The phenomenon is observed in diseases of the extrapyramidal system and is caused by active tension of the muscles due to the approximation of their attachment points; it represents one of the forms of the so-called "reflexes of posture" (reflexes de posture) that are now being intensively studied. 3. Pseudostupor-like state due to affective delusional ideas and hallucinations. Westphal's syndrome consists of periodic (paroxysmal) paralysis of the limbs. Usually a familial disease (auto-intoxication?), in which transient flaccid paralysis of the muscles of the limbs and trunk occur in attacks, with intervals from one day to several months. Muscles innervated by cranial nerves, especially facial and oculomotor, are as a rule not affected in this condition. Excitability to both electric currents, as well as mechanical excitability, is reduced or completely lost during an attack. Tendon reflexes in the affected limbs are weakened or absent, while cutaneous reflexes are normal. The duration of attacks is usually several hours. Between attacks, patients feel completely healthy.

I. Prislany. (K. Westphal, Strumpell), pseudosclerosis, a disease first described by Westphal (1883) and later by Strümpel (1898). Previously it was often classified in the group of neuroses; at present, on the basis of the clinical picture and patho-anatomical findings, it is classified in the large group of diseases of the extrapyramidal system. Etiology is completely obscure. The disease usually develops in adolescence and often has a familial character. Direct inheritance is noted as a rare exception. Anatomically it is characterized by a peculiar gliosis, especially the appearance of giant glial cells (Alzheimer), which are found, mainly, in the striatum, but also in the thalamus, in the regio subthalamica, in the pons Varoli, in the nucleus dentatus and (in small numbers) in the cortex. Essentially, it is a lesion of the entire central nervous system, although with special emphasis on the basal ganglia. Typical Alzheimer cells are giant glial elements with enormous lobulated nuclei. A very characteristic patho-anatomical finding is cirrhosis of the liver. Symptomatology: rhythmic hyperkinesia of large amplitude - throwing, voluminous involuntary movements, especially in the large joints. Tone is usually altered - sometimes typical extrapyramidal rigidity, more often hypotonia. Poverty of movement, especially some degree of amimia. Speech is most often of the bulbar type or scanned. Often disturbance of swallowing and epileptoid attacks. Almost always phenomena of organic dementia. As a very characteristic sign, described by Kaiser and Fleischer (Kayser, Fleischer) - the pericorneal ring (a narrow, olive-colored pigmented ring at the edge of the cornea). Changes in liver function are noted. As the very name (pseudosclerosis) indicates, its authors found similarity with disseminated sclerosis. This similarity, however, concerns only certain symptoms. In pseudosclerosis, such signs as nystagmus, atrophy of the optic nerve, disappearance of abdominal reflexes, pathological reflexes (they are described in pseudosclerosis only in rare cases) are absent. In disseminated sclerosis there is no pericorneal ring, cirrhosis of the liver, extrapyramidal disorders. Very great difficulties are encountered in the differential diagnosis from Wilson's disease. To a certain extent, the difference is the hyperkinesis; in Wilson's disease there is always rigidity; the pericorneal ring is not typical. But all these signs have only very conditional significance, there are many transitional cases, and both forms can be classified in one nosological group (see Wilson's disease). The course is chronic, progressive, sometimes with small remissions. The prognosis is hopeless. Therapy is so far without result.