Language Related Disorders

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APHEMIA

Aphemia was the term originally used by Broca to describe all language disturbances, but he later accepted the term aphasia promoted by Trousseau (1864). Bastian (1887) used aphemia to denote a specific syndrome described here; others have termed the same language abnormality pure motor aphasia, cortical anarthria, or apraxia of speech.

The patient presents acutely with mutism but is able to communicate using written language. When verbalization returns, the output is hypophonic, slow, and poorly articulated so as to be almost incomprehensible. Comprehension of spoken language is intact. Although both repetition and naming are severely disturbed because they demand vocalization, patients can comprehend written language and are able to write without difficulty. Primary laryngeal pathology should be ruled out.

Neurological examination reveals a transient right hemiplegia or hemiparesis without other neurological signs except for an often associated buccofacial apraxia. Patients have difficulty performing on command acts such as whistling, sucking, blowing, and winking. In contrast, they have no trouble performing limb activities such as imitating the use of a comb or a toothbrush, waving goodbye, and saluting.

In aphemia, the communication disturbance involves verbal output and is a speech problem rather than an impairment of language. There is often an overlap, however, between aphemia and the anterior aphasia syndromes. An aphemia-like state appears in some patients who have recovered from Broca's aphasia. Some investigators have even considered aphemia to be a minor subtype of Broca's aphasia, also called little Broca's aphasia.

However, aphemia differs from true Broca's aphasia because of the absence of agrammatism in the spoken and written output. Most often, aphemia improves to useful language output, but a dysprosodic speech quality remains, producing a so-called foreign accent syndrome, a disorder of rhythm, inflection, and articulation, suggesting that the patient is not a native speaker of the language. The pathology is located in or immediately inferior to Broca's area in the dominant hemisphere (see Fig. 6,-5 ).[gi

TABL

E 6-5 -- CLINICO-ANATOMICAL CORRELATIONS OF SPEECH AND LANGUAGE DISORDERS

Anatomical Site of Damage

Speech or Language Syndrome

Other Neurological and Medical Findings

Common Etiologies

Comments

(L) Posterior inferior frontal lobe

Broca's aphasia

Brachiofacial weakness

Stroke, trauma, tumor, infection

Initial (L) conjugate gaze deviation

(L) Posterior superior temporal gyrus

Wernicke's aphasia

Visual field deficit

Stroke, temporal lobe abscess

(L) Posterior sylvian region

Conduction aphasia

Cortical sensory deficit pseudothalamic pain

Stroke

Associated ideomotor apraxia

(L) Sylvian region

Global aphasia

Hemianopia, hemiplegia

Stroke, tumor, trauma, infection

(L) Dorsolateral frontal cortex

Transcortical motor aphasia

Buccofacial apraxia, hemiparesis is occasional

Anterior border zone vascular infarction

(L) Temporoparieto-occipital junction

Transcortical sensory aphasia

Cortical sensory deficit Visual field deficit

Posterior border zone vascular infarction

Degenerative disease

Echolalia is common

(L) Hemisphere, vascular border zone areas

Mixed transcortical aphasia

Variable motor, sensory, and visual field deficits

Border zone infarctions, anoxia, carbon monoxide poisoning

Echolalia very common

(L) Thalamus, striatum white matter

Subcortical aphasia

Hemiplegia/sensory loss

Stroke, ischemic and hemorrhagic

No visual field or cortical sensory loss

Unilateral or bilateral corticobulbar tracts

Spastic dysarthria

Unilateral or bilateral hyperreflexia or spasticity

Stroke, demyelinating and degenerative disease

Cerebellum

Ataxic dysarthria

Dysmetria, nystagmus ataxia

Stroke, degenerative, toxic disorder; tumor

Lower motor neuron brain stem or peripheral nerves

Flaccid dysarthria

Atrophy, fasciculation, decreased gag

Motor neuron disease, stroke, carcinomatous meningitis

Risk of aspiration pneumonia

Basal ganglia

Hypokinetic/hyperkinetic dysarthria

Parkinsonism, dystonia, chorea

Degenerative disease, Parkinson's disease, Huntington's disease, stroke

Vocal cords or their innervation

Dysphonia

Fatiguability, ptosis

Laryngitis, vocal cord paralysis, myasthenia

May be a sign of laryngeal carcinoma

PURE WORD DEAFNESS

Pure word deafness is a rare prelanguage syndrome in which only reception of spoken language is involved. Individuals with pure word deafness do not understand spoken language and cannot repeat. However, they are able to hear and identify adequately nonverbal sounds such as a whistle, a telephone ring, and a dog bark. Their reading is intact, and they often carry with them a writing tablet for others to use. y

There are two known loci of pathology. Some patients have a single lesion deep in the dominant superior temporal region affecting the primary auditory cortex or the pathways to it from the medial geniculate nucleus. More commonly, there is bilateral pathology involving the mid-portion of the superior temporal gyrus of both hemispheres. Wernicke's area (left auditory association cortex) is not involved.

Some cases of pure word deafness have associated paraphasic output and mild written language comprehension deficits. These cases should be classified as Wernicke's aphasias with predominant word deafness and relatively less word blindness (reading comprehension).

Pure word deafness is not a disturbance of language ability. Delivery of the auditory signal to the area mediating language interpretation is defective, which is a sensory transmission problem rather than a language disturbance. Pure word deafness resembles deafness more than aphasia. It has also been called auditory verbal agnosia.y Although pure word deafness is rare in isolation, it may be associated with posterior aphasic disorders.

DELIRIUM

Delirium with fluctuating consciousness and disturbed thought content is associated with variable language difficulties with or without structural brain pathology. Patients often have impaired attention and do poorly on tasks such as digit span and mental control. Language disturbances can be the presenting feature of delirium, particularly in patients with decreased attention and concentration. Associated features may include agitation, hallucinations, confusion, and myoclonus.

PSYCHIATRIC DISORDERS

Psychiatric disorders can occasionally mimic classic language syndromes. Schizophrenics often present with verbal output described as word salad, containing bizarre words and sentences. Written output parallels the content of verbal output. These patients have no other language disturbances and are usually readily distinguished from Wernicke's or other posterior aphasias. Manic patients often present with logorrheic output, pressured speech, and grandiose content; but no other language disturbances are

encountered that distinguish them from other classic aphasia syndromes. RIGHT HEMISPHERE DISORDERS

Right hemisphere disorders can affect language function, most commonly in left-handed individuals. Rarely, right-handed patients become aphasic after strokes in the right hemisphere, a phenomenon called crossed aphasia in dextrals. Even in right-handed people with typical left hemisphere dominance for language, subtle alterations in language function occur after right hemisphere damage. The right hemisphere has a dominant role for expressing emotion including emotional prosodic aspects of communication.^ Amelodia (also called affective motor aprosodia) is a disturbance characterized by loss of melody, prosody, or emotional intonation in verbal output.y There is a flat monotonous verbal output, inability to produce a melody when singing (expressive amusia), decreased facial expression, and sparse use of gestures. The result is a seemingly emotionless response, which is easily misinterpreted as depression. The neuropathology involves the right frontal opercular area and its connections, which is the right hemisphere equivalent of Broca's area. In addition, receptive amusia and other disorders of music appreciation are related disturbances predominantly associated with right hemisphere lesions.

Tables 6-5 and 6-6 summarize clinico-anatomical correlations

TABLE 6-6 -- SELECTED ETIOLOGIES OF SPEECH AND LANGUAGE DISORDERS

Etiological Category

Selected Specific Etiologies

Chapter

STRUCTURAL DISORDERS

Developmental

Arnold-Chiari malformat ons

28

HEREDITARY AND DEGENERATIVE DISORDERS

Storage diseases: Lipidoses, glycogen disorders and leukoencephalopathies

Adrenoleueodystrophy, Krabbe's disease, metachromatic leucodystrophy

30

The degenerative dementias

Alzheimer's disease, fronto-temporal degeneration, Parkinson's disease, Huntington's disease

33

Movement disorders

Hallervorden-Spatz disease, Wilson's disease, progressive supranuelear palsy, Parkinson's disease, multiple system atrophy, Huntington's disease

34

Ataxias

Friedreich's ataxia, abetalipoproteinemia

35

Degenerative motor, sensory, and autonomic disorders

Amyotrophic lateral sclerosis

36

Inherited muscle, neuromuscular, and neuronal disorders

Myopathies

37

ACQUIRED METABOLIC AND NUTRITIONAL DISORDERS

Endogenous metabolic disorders

Hypothyroidism

38

Exogenous aequired metabolic disorders of the nervous system: Toxins and illicit drugs

Cocaine

39

Nutritional deficiencies and syndromes associated with alcoholism

Alcoholic cerebellar degeneration Vitamin E deficiency

40

INFECTIOUS DISORDERS

Viral infections

Herpetic encephalitis

41

Nonviral infections

Toxoplasmosis or bacterial abscess of the temporal lobe

42

Transmissible spongiform encephalopathies HIV and AIDS

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