PATIENT NO. 1
A 42-year-old right-handed graduate lady presented with:
- Recurrent episodes of right-sided limb weakness for two months.
- Speaking difficulty for two months.
She had four discrete episodes of weakness. Initially, two episodes over two consecutive days, and later two more episodes.
During her initial two episodes, she developed sudden onset of distal right upper limb weakness with heaviness in the right leg which progressed over five minutes and lasted for 10 minutes. There was no history of speech or visual symptoms and the weakness improved completely.
One and half months later, she was apparently normal and doing her routine work in the morning, when suddenly she developed difficulty in speaking. Her word output reduced to one or two and she started communicating with gestures. She was able to comprehend the spoken word but could not read and write. She could not name persons and objects around her. There was no limb and visual symptoms. The speech disturbances were gradually improving when she presented but still she was facing difficulty in conversing due to difficulty in naming objects and persons, even though her comprehension was normal.
Two days prior to her presentation to our hospital, she again developed sudden onset of mild right-sided upper limb more than lower limb weakness without new speech or visual symptoms. The weakness progressed over 15–20 minutes, then started improving.
No visual symptoms were associated with any of these recent episodes.
She was known hypertensive from the past 5 years on regular treatment. She had experienced 5–6 attacks of transient blurring of vision in her left eye lasting for few minutes recovering spontaneously fully over 15 minutes one year prior to her present illness.
On Examination
She was moderately built and nourished. Pulse rate—76/minute, regular, reduced volume in left carotid artery. No bruits. Blood pressure in right upper limb 180/90 mm Hg, left upper limb 160/90 mm Hg. Cardiac and respiratory system examination were normal.
- Handedness—Right-handed
- Education—Graduate
Neurological Examination
Minimental status score (MMSE): 27/30.
Language
- Spontaneous speech: Reduced fluency (category and verbal) and word output, uses few words to answer rather than sentences, speech was effortful, occasional paraphasias were present, no neologisms. Prosody normal, no dysarthria.
- Comprehension: Answers Yes and No to questions and pointing to the objects correctly.
- Writing: Writing spontaneously, writing in response to command to copy and to dictation were impaired.
- Repetition: Could repeat sentences up to 4–5 words.
- Naming: Could name objects, colors, etc. but had difficulty in naming parts of the objects.
- Reading: Could read few words but could not read continuously.
Cranial Nerves
- Visual acuity right side 6/9, left side 6/12 with right-sided hemianopia.
- Pupillary reaction optic fundus and eye movements were normal.
- Right-sided upper motor neuron (UMN) facial palsy was seen.
- Other cranial nerves normal.
- Motor system: Right elbow and wrist extension 4/5, rest of the power was normal.
- Sensory system: Normal.
Summary
This middle-aged lady with 5 years history of hypertension presented with episodes of left monocular visual loss and reduced carotid volume suggestive of left carotid artery disease with recent symptoms of motor and language disturbances in the left-sided carotid/middle cerebral artery territory. She had motor weakness pyramidal in nature localizing above the pons and language disturbance in the form of Motor (Broca's) aphasia localizing to left frontal motor speech area. In her repetition was also involved suggestive of cortical localization of the language disturbances.
PATIENT NO. 2
A 42-year-old lady with no formal education, right-handed presented with history of headache of 15 days and speech disturbances from the past 7 days.
Headache was acute in onset, continuous, throbbing, bifrontal, associated with vomiting, no photophobia or phonophobia, or visual disturbances, not relieved with analgesics associated with difficulty in doing work.
Eight days after the onset of headache she suddenly developed speech disturbances. Relatives noticed that she was not able to understand the spoken words and she was speaking but nobody could understand the meaning of the sentences. She was calling her relatives by different words which had no meaning, was speaking irrelevantly and they could not make out meaning from her speech.
No history of loss of consciousness, visual symptoms, weakness of limbs, seizures or sensory disturbances.5
No history of ear discharge, fever, trauma, menstrual irregularities or consumption of hormonal preparations.
On Examination
General physical examination: She had mild pallor. Blood pressure, cardiac, abdominal and respiratory systems were normal.
Neurological Examination
She was right-handed, with no formal education. She was conscious, able to walk around.
Minimental status examination (MMSE): Could not be assessed because of her language disturbances.
Language Assessment
- Spontaneous speech: It was fluent, effortless, with paraphasic errors, speech had no meaning. She could not speak about her personal details (name, address, occupation), and she speaks irrelevantly, could not describe a picture.
- Comprehension: Severely impaired with inability to point to said object and could not answer to y and n questions.
- Repetition: Could not understand the commands.
- Naming: Not able to do.
- Reading: Could not be assessed.
- Writing: Could not write her name.
Fundus was normal no papilledema, visual acuity was grossly normal with right-sided hemianopia.
Mild right-sided UMN facial nerve palsy.
Motor, sensory and cerebellar system: Normal.
Summary
Middle aged lady with no premorbid diseases presented with subacute onset of new headache with raised intracranial tension. One week later, she developed language disturbances suggestive of sensory or Wernicke's aphasia with mild UMN facial palsy and right-sided hemianopia localizing to left temporoparietal region of brain. On further evaluation she was found to have left temporoparietal venous infarct due to cerebral venous thrombosis.
Q.1. Define language and speech and in what way their disturbances manifest?
Ans. Language is defined as complex system of communication with symbols and sounds and it also has rules for their use. Speech is defined as articulation and phonation of language sounds and it is a part of language.
The disturbances of language are called as aphasias, which means loss or impairment of production or comprehension of spoken or written language secondary to acquired lesion of the brain.
Disorders of speech includes dysarthria (defective articulation with intact mental functions and normal syntax), dysphonia (alteration or loss of voice due to laryngeal disorder or its innervation), and stuttering.6
Q.2. Where are the centers for language located and what is their function and blood supply to the areas?
Ans. There are four main language areas (Perisylvian Area): 2 receptive and 2 executive which are situated in the left hemisphere in right-handed person:
- Reception: It is situated in the posterior superior temporal gyrus (area 22) and Heschl's gyri (area 41 and 42). Posterior part of area 22 in planum temporale is Wernicke's area (Blood supply is by inferior division of the left middle cerebral artery).
- Receptive area: Angular gyrus (area 39) in inferior parietal lobule, anterior to visual receptive areas.
- Execution: Posterior end of inferior frontal convolution (area 44 and 45), called as Broca's area (Blood supply is by upper division of left middle cerebral artery).
- Exner's writing area—posterior end of 2nd frontal convolution.
Q.3. What is the normal distribution of handedness in general population? How it is deter- mined?
Ans. About 90–95% general population are right-handed (they innately choose right hand).
Edinburgh handedness inventory: This inventory determines the handedness by assessing which hand is used for various daily routine tasks (10 types like: writing, drawing, throwing a ball, scissor, toothbrush, knife, spoon, broomstick, strike a matchbox, open a box lid). Each task is given a score and a final score is generated which suggests the handedness.
Determining handedness: Ask which hand is preferred for throwing a ball, threading a needle, sewing, using a tennis racket or hammer, which eye is used for sighting a target with a rifle or looking through a key-hole, telescope, etc. (Eye preference coincides with hand preference).
Q.4. What are the features of Broca's aphasia?
Ans. Patients with Broca's or motor aphasia have nonfluent spontaneous speech, which is telegraphic, dysarthric or mute, impaired naming, reading and repetition, writing may be dysmorphic or dysgrammatic, can have associated right hemiparesis, right hemisensory loss or even apraxia of left limbs. Comprehension will be normal.
Q.5. What are the features of Wernicke's aphasia?
Ans. The spontaneous speech will be fluent with paraphasic errors, usually speech will be non-dysarthric and can be logorrheic. Comprehension is impaired with difficulty in pointing things and answering to yes and no questions. Naming, reading writing and repetition are impaired. Can have associated right hemianopia. In pure Wernicke's aphasia motor and sensory signs are usually minimal or absent.
Q.6. What are the features of global aphasia?
Ans. Patients are usually mute or have nonfluent spontaneous speech, impaired naming, comprehension, repetition, reading and writing. They have associated dense right hemiparesis, hemisensory loss and right hemianopia.
Q.7. What is anomia agraphia and alexia?
Ans.
- Anomia is impaired naming
- Agraphia is impaired writing
Q. 8. What is dysarthria and its various types?
Ans. Dysarthria is abnormal articulation of sounds or phonemes. Total loss of ability to articulate is anarthria.
Mayo clinic classification of dysarthria (Duffy, 1995)—6 types:
- Flaccid: Lower motor neuron, bulbar weakness, myasthenia.
- Spastic: Bilateral upper motor neuron (or) unilateral UMN—strokes, tumors, Primary lateral sclerosis.
- Ataxic: Cerebellar diseases (stroke, degenerative disease).
- Hypokinetic: Extrapyramidal—Parkinson's disease.
- Hyperkinetic: Extrapyramidal—Dystonia, Huntington's disease.
- Mixed-spastic and flaccid: Upper and lower motor neuron involvement—Amyotrophic lateral sclerosis, multiple strokes.
Q.9. How speech and language disorders are treated?
Ans. Treatment of Aphasia
- Client-specific behaviors are targeted.
- Target greatest improvement in functional communication.
- Start from simple to more complex tasks.
- Reinforce the response—give feedback.
- Teach self-monitoring skills.
- Train spouse/relative to evoke, prompt and support the patient.
Majority improve spontaneously (in days, weeks, months) (Neuronal plasticity). Other methods reassurance, speech rehabilitation. Prognosis better for left-handed persons.
Treatment of Speech Disorders
Depends on type of dysarthria and natural history of the disease:
- Pacing devices, palatal lifts, communication boards, visual cues, pharyngeal flap, vocal cord teflon injection.
- Speech therapy, psychotherapy.
- Botulinum toxin injection (for spasmodic dysphonia).
- For stuttering—Behavioral techniques, altered auditory feedback, pharmacotherapy-risperidone, olanzapine, paroxetine.